MedWorm: Hairy Cell Leukemia

Correlation of the BRAF V600E mutation in hairy cell leukaemia with morphology, cytochemistry and immunophenotype

Clinical and Laboratory Haematology — Wed, 01 Feb 2012 05:00:00 +0100

SummaryHairy cell leukaemia (HCL) has distinct clinical, morphological and immunophenotypic features with no recurrent cytogenetic or molecular abnormalities reported until the recent description of the BRAF V600E mutation in patients with classical HCL. The incidence of this mutation was sought in 27 patients with either classical HCL or HCL variant by an allele‐specific PCR approach and findings related to morphology, cytochemistry and immunophenotype. A high degree of correlation was noted between the presence of BRAF V600E and established diagnostic criteria in 26/27 patients with HCL/HCL variant. Detection of the BRAF V600E mutation is therefore a useful adjunct in the differential diagnosis of HCL and HCL variant and highlights the value of a multifaceted approach to the diagnosis ...

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Case report of hairy cell leukemia

Jornal Brasileiro de Patologia e Medicina Laboratorial — Sat, 14 Jan 2012 16:50:49 +0100

The objective of this study was to report the case of a male patient, tractor operator, diagnosed with HCL, pancytopenia, cutaneous lesions, without splenomegaly and positive markers for B-cell lymphocytes (CD19, CD20, CD22, CD79b, CD23, Lambda, immunoglobulin M [IgM], CD25 and CD103). Although HCL is a rare disease, late diagnosis may ultimately lead to severe complications and patient's death. (Source: Jornal Brasileiro de Patologia e Medicina Laboratorial)

Langerhans/dendritic cell sarcoma arising from hairy cell leukemia: a rare phenomenon

Annals of Hematology — Wed, 11 Jan 2012 06:57:54 +0100

Content Type Journal ArticleCategory Letter to the EditorPages 1-3DOI 10.1007/s00277-011-1399-5Authors Alaa Muslimani, Department of Hematology and Oncology, Oakland University William Beaumont School of Medicine, William Beaumont Hospital, 3577 W 13 Mile Rd, Suite 202, Royal Oak, MI 48073, USAMohammad Muhsin Chisti, Department of Hematology and Oncology, Oakland University William Beaumont School of Medicine, William Beaumont Hospital, 3577 W 13 Mile Rd, Suite 202, Royal Oak, MI 48073, USAAnn Marie Blenc, Department of Pathology, Oakland University William Beaumont School of Medicine, William Beaumont Hospital, 3601 W 13 Mile Rd, Royal Oak, MI 48073, USAIqbal Boxwala, Department of Hematology and Oncology, Oakland University William Beaumont School of Medicine, William Beaumont Hospit...

The BRAF V600E mutation in hairy cell leukemia and other mature B-cell neoplasms

Blood — Thu, 05 Jan 2012 05:00:00 +0100

The somatically acquired V600E mutation of the BRAF gene has been recently described as a molecular marker of hairy cell leukemia (HCL). We developed an allele-specific PCR for this mutation and studied 62 patients with HCL, 1 with HCL variant, 91 with splenic marginal zone lymphoma, 29 with Waldenström macroglobulinemia, and 57 with B-cell chronic lymphoproliferative disorders. The BRAF V600E mutation was detected in all HCL cases and in only 2 of the remaining 178 patients. These 2 subjects had B-cell chronic lymphoproliferative disorders that did not fulfill the diagnostic criteria for HCL. Despite the positive PCR finding, the mutation could not be detected by Sanger sequencing in these 2 cases, suggesting that it was associated with a small subclone. We conclude that the BRAF V60...

Simple genetic diagnosis of hairy cell leukemia by sensitive detection of the BRAF-V600E mutation

Blood — Thu, 05 Jan 2012 05:00:00 +0100

In this study, we describe a new, simple, and inexpensive test for genetics-based diagnosis of HCL in whole-blood samples that detects BRAF-V600E through a sensitive allele-specific PCR qualitative assay followed by agarose-gel electrophoresis. This approach detected BRAF-V600E in all 123 leukemic HCL samples investigated containing as few as 0.1% leukemic cells. BRAF-V600E was detected at different time points during the disease course, even after therapy, pointing to its pivotal role in HCL pathogenesis and maintenance of the leukemic clone. Conversely, 115 non-HCL chronic B-cell neoplasms, including 79 HCL-like disorders, were invariably negative for BRAF-V600E. This molecular assay is a powerful tool for improving the diagnostic accuracy in HCL. (Source: Blood)

Langerhans cell sarcoma in a patient with hairy cell leukemia: common clonal origin indicated by identical immunoglobulin gene rearrangements.

Journal of Cutaneous Pathology — Sun, 01 Jan 2012 05:00:00 +0100

(Source: Journal of Cutaneous Pathology)

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Hairy Cell Leukemia Variant, a New Entity of the WHO 2008 [DIAGNOSIS IN ONCOLOGY]

Journal of Clinical Oncology — Fri, 16 Dec 2011 05:00:00 +0100

(Source: Journal of Clinical Oncology)

The cytotoxicity of anti-CD22 immunotoxin is enhanced by bryostatin1 in B-cell lymphomas through CD22 up-regulation and PKC-βII depletion.

Haematologica — Fri, 16 Dec 2011 05:00:00 +0100

Conclusions:Our data suggest that the combination of Bryostatin1 with antibodies directed against CD22 is a potent drug combination for the treatment of low and high-grade B-cell lymphoma. PMID: 22180432 [PubMed - as supplied by publisher] (Source: Haematologica)

Pyrosequencing of BRAF V600E in routine samples of Hairy Cell Leukaemia identifies CD5+ variant Hairy Cell Leukaemia that lacks V600E

British Journal of Haematology — Wed, 07 Dec 2011 05:00:00 +0100

(Source: British Journal of Haematology)

Detection of BRAF mutations in patients with hairy cell leukemia and related lymphoproliferative disorders.

Haematologica — Thu, 01 Dec 2011 05:00:00 +0100

Authors: Blombery P, Wong SQ, Hewitt CA, Dobrovic A, Maxwell EL, Juneja S, Grigoriadis G, Westerman DA Abstract Hairy cell leukemia has been shown to be strongly associated with the BRAF V600E mutation. We screened 59 unenriched archived bone marrow aspirate and peripheral blood samples from 51 patients with hairy cell leukemia using high resolution melting analysis and confirmatory Sanger sequencing. The BRAF V600E mutation was detected in 38 samples (from 36 patients). The BRAF V600E mutation was detected in all samples with disease involvement above the limit of sensitivity of the techniques used. 33/34 samples from other hematological malignancies were negative for BRAF mutations. A BRAF K601E mutation was detected in a patient with splenic marginal zone lymphoma. Our data supp...

Real‐time PCR‐based analysis of BRAF V600E mutation in low and intermediate grade lymphomas confirms frequent occurrence in hairy cell leukaemia

Hematological Oncology — Thu, 01 Dec 2011 05:00:00 +0100

AbstractHairy cell leukaemia (HCL) is a rare type of B‐cell non‐Hodgkin lymphoma (B‐NHL), which is not known to be associated with any characteristic recurrent karyotypic abnormality. A recent study that used massively parallel whole exome sequencing identified an activating V600E mutation in BRAF, which appeared specific for HCL. Here, we confirm the specificity of BRAF V600E for HCL among low and intermediate grade B‐NHL and describe a real‐time polymerase chain reaction method for detecting this mutation in cases with low tumour burden. The V600E mutation does not appear to be associated with microsatellite instability, unlike the case in colorectal cancer. Thus, in conjunction with prior data, our results suggest incorporation of BRAF V600E mutation analysis in the diagnostic...

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Revised guidelines for the diagnosis and management of hairy cell leukaemia and hairy cell leukaemia variant*

British Journal of Haematology — Thu, 24 Nov 2011 05:00:00 +0100

SummaryThe British Committee for Standards in Haematology first produced guidelines for the diagnosis and management of hairy cell leukaemia and hairy cell leukaemia variant in 2000. This revision updates those guidelines and covers the areas of diagnosis, treatment and assessment of response to therapy. (Source: British Journal of Haematology)

Chronic Lymphocytic Leukemia and Hairy-Cell Leukemia

Cancer Network — Wed, 09 Nov 2011 12:00:00 +0100

This management guide covers the epidemiology, etiology and risk factors, signs and symptoms, laboratory features, cytogenetic and molecular findings, staging and prognosis, and treatment of chronic lymphocytic leukemia, as well as the treatment of hairy-cell leukemia. (Source: Cancer Network)

Synergistic antitumor activity of anti-CD25 recombinant immunotoxin LMB-2 with chemotherapy.

Clinical Cancer Research — Tue, 08 Nov 2011 05:00:00 +0100

CONCLUSIONS: Gemcitabine is synergistic with LMB-2 in vivo unrelated to improved cytotoxicity. Synergism therefore appears related to improved distribution of LMB-2 to CD25+ tumors, and is preceded by decreased sCD25 within the tumor due to chemotherapy. To test the concept of combined treatment clinically, patients with relapsed/refractory adult T-cell leukemia are being treated with fludarabine plus cyclophosphamide prior to LMB-2. PMID: 22068660 [PubMed - as supplied by publisher] (Source: Clinical Cancer Research)

Fc receptor-like 5 promotes B cell proliferation and drives the development of cells displaying switched isotypes.

Journal of Leukocyte Biology — Tue, 25 Oct 2011 04:00:00 +0100

Authors: Dement-Brown J, Newton CS, Ise T, Damdinsuren B, Nagata S, Tolnay M Abstract The biological roles of B cell membrane proteins in the FCRL family are enigmatic. FCRL proteins, including FCRL5, were shown to modulate early BCR signaling, although the subsequent, functional consequences of receptor engagement are poorly understood. We found that FCRL5 surface protein itself was induced temporarily upon BCR stimulation of human, naive B cells, indicating precise control over timing of FCRL5 engagement. Cross-linking of FCRL5 on cells induced to express FCRL5 enhanced B cell proliferation significantly. This enhancement required costimulation of the BCR and TLR9, two signals required for optimal proliferation of naive B cells, whereas T cell help in the form of anti-CD40 and IL...

Antibody Fusion Proteins: Anti-CD22 Recombinant Immunotoxin Moxetumomab Pasudotox.

Clinical Cancer Research — Sat, 15 Oct 2011 04:00:00 +0100

Authors: Kreitman RJ, Pastan I Abstract Recombinant immunotoxins are fusion proteins that contain the cytotoxic portion of a protein toxin fused to the Fv portion of an antibody. The Fv binds to an antigen on a target cell and brings the toxin into the cell interior, where it arrests protein synthesis and initiates the apoptotic cascade. Moxetumomab pasudotox, previously called HA22 or CAT-8015, is a recombinant immunotoxin composed of the Fv fragment of an anti-CD22 monoclonal antibody fused to a 38-kDa fragment of Pseudomonas exotoxin A, called PE38. Moxetumomab pasudotox is an improved, more active form of a predecessor recombinant immunotoxin, BL22 (also called CAT-3888), which produced complete remission in relapsed/refractory hairy cell leukemia (HCL), but it had a <20% re...

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Phase 2 study of cladribine followed by rituximab in patients with hairy cell leukemia

Blood — Thu, 06 Oct 2011 04:00:00 +0100

This study was registered at www.clinicaltrials.gov as NCT00412594. (Source: Blood)

Editorial: a defining moment for hairy cell leukaemia

British Journal of Haematology — Mon, 19 Sep 2011 04:00:00 +0100

(Source: British Journal of Haematology)

Characteristic CD103 and CD123 Expression Pattern Defines Hairy Cell Leukemia: Usefulness of CD123 and CD103 in the Diagnosis of Mature B-Cell Lymphoproliferative Disorders.

American Journal of Clinical Pathology — Fri, 16 Sep 2011 19:46:21 +0100

Authors: Venkataraman G, Aguhar C, Kreitman RJ, Yuan CM, Stetler-Stevenson M Abstract By using flow cytometry, we studied CD103 and CD123 expression by the malignant cells in 300 B-cell lymphoproliferative disorder (BC-LPD) cases, including 114 hairy cell leukemia (HCL), 20 HCL variant (HCLv), 9 splenic marginal zone lymphoma (SMZL; in 5, only CD103 was evaluated), 133 chronic lymphocytic leukemia (CLL), 3 follicular lymphoma (FL), and 21 mantle cell lymphoma (MCL). All HCLs expressed uniform CD103 and bright CD123. Among the 20 HCLv cases, 20 (100%) were CD103+ and 8 (40%) were CD123+ (partial or dim). CD103 was negative in all MCL, FL, CLL, and SMZL cases. CD123 was positive in 1 (25%) of 4 SMZL, 3.8% of CLL (5/133), 7 (33%) of 21 MCL, and 1 (33%) of 3 FL cases. CD103 is specific...

High resolution melting analysis for detection of BRAF exon 15 mutations in hairy cell leukaemia and other lymphoid malignancies

British Journal of Haematology — Tue, 13 Sep 2011 04:00:00 +0100

SummaryThe BRAF V600E mutation has recently been described in all cases of hairy cell leukaemia (HCL). We have developed and validated a rapid and sensitive high‐resolution melting analysis (HRMA) assay that detects BRAF exon 15 mutations when hairy cells are as low as 5–10% in a sample. All 48 HCL patients were positive for the BRAF V600E mutation, while 114 non‐HCL cases were all V600E negative. Interestingly, we detected a novel BRAF D594N mutation in one patient with multiple myeloma. The HRMA assay offers a useful tool to aid the laboratory diagnosis of HCL. (Source: British Journal of Haematology)

BRAF Mutations in Hairy-Cell Leukemia

New England Journal of Medicine — Wed, 07 Sep 2011 21:00:11 +0100

New England Journal of Medicine, Volume 365, Issue 10, Page 960-962, September 2011. (Source: New England Journal of Medicine)

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TrovaGene, Inc., Announces Worldwide Exclusive Licensing of Assay for Hairy Cell Leukemia

HSMN NewsFeed — Wed, 07 Sep 2011 12:48:37 +0100

SAN DIEGO--(Healthcare Sales & Marketing Network)-- TrovaGene, Inc. (Pink Sheets:TROV.pk ), a developer of transrenal molecular diagnostics to facilitate personalized medicine, has signed a worldwide exclusive license for an assay that detects Hairy Cell L... Diagnostics, Licensing, OncologyTrovaGene, Hairy Cell Leukemia, BRAF gene (Source: HSMN NewsFeed)

Synchronous Detection of Hairy Cell Leukemia and HIV-Negative Kaposi’s Sarcoma of the Lymph Node: A Diagnostic Challenge and a Rare Coincidence

Karger Publishers — Fri, 02 Sep 2011 23:00:00 +0100

Case Rep Oncol 2011;4:439–444 (DOI:10.1159/000331894) (Source: Karger Publishers)

Differential and tumor-specific expression of CD160 in B-cell malignancies

Blood — Wed, 24 Aug 2011 23:00:00 +0100

CD160 is a human natural killer (NK)-cell–activating receptor that is also expressed on T-cell subsets. In the present study, we examined 811 consecutive cases of B-cell lymphoproliferative disorders (B-LPDs), and demonstrated CD160 expression in 98% (590 of 600) of chronic lymphocytic leukemia (CLL) cases, 100% (32 of 32) of hairy cell leukemia (HCL) cases, 15% (5 of 34) of mantle cell lymphoma (MCL) in the leukemic phase, and 16% (23 of 145) of other B-LPD cases. CD160 transcript and protein were absent in the normal B-cell hierarchy, from stem cells, B-cell precursors, maturing B cells in the germinal center, and circulating B cells, including CD5+CD19+ B1 cells in umbilical cord. CD160 positivity was significantly higher in CLL and HCL in terms of percentage (65.9% and 67.8%, res...

An assessment of the usefulness of immunohistochemical stains in the diagnosis of hairy cell leukemia.

American Journal of Clinical Pathology — Mon, 22 Aug 2011 21:45:03 +0100

Authors: Sherman MJ, Hanson CA, Hoyer JD Abstract Annexin-1 and T-bet are recently described immunohistochemical stains that reportedly assist in the diagnosis of hairy cell leukemia (HCL). Our objective was to assess the sensitivity and specificity of a panel of immunohistochemical stains in distinguishing HCL from other B-cell neoplasms, particularly splenic and extranodal marginal zone lymphomas (SMZL and ENMZL, respectively). The study included 234 bone marrow biopsy specimens: 101 HCL, 13 SMZL, and 10 ENMZL cases were assessed with CD20, tartrate-resistant acid phosphatase (TRAP), DBA.44, a-1, T-bet, and cyclin D1, and 110 control cases were assessed with annexin-1 and T-bet. Our study showed that annexin-1 is a specific and sensitive marker for HCL; however, interpretation is...

The diagnostic value of CD1d expression in a large cohort of patients with B-cell chronic lymphoproliferative disorders.

American Journal of Clinical Pathology — Mon, 22 Aug 2011 21:44:47 +0100

Authors: Kotsianidis I, Nakou E, Spanoudakis E, Bouchliou I, Moustakidis E, Miltiades P, Vadikolia CM, Szydlo R, Karadimitris A, Tsatalas C Abstract Immunophenotyping is indispensable in the differential diagnosis of B-cell chronic lymphoproliferative disorders (B-CLPDs). However, B-CLPDs often show overlapping immunophenotypic profiles and may be diagnostically challenging. CD1d is an HLA class I-like molecule that presents glycolipids to invariant natural killer T cells. Normal mature B cells constitutively express CD1d, but with the exception of some conflicting data, its expression in B-CLPDs is unknown. We demonstrate that in 222 B-CLPD cases, CD1d expression of less than 45% is strongly predictive of CLL (likelihood ratio, 32.3; specificity, 97.4%; sensitivity, 84.1%). In add...

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Classical Hairy Cell Leukemia and Its Variant: A 17-Year Retrospective Survey in Taiwan Chinese.

Acta Haematologica — Fri, 12 Aug 2011 23:00:00 +0100

Conclusion: A relatively higher proportion of HCL-V in all HCL comparing to Westerners is observed. Second malignancies are common. With an inferior outcome and dismal response to most treatment, enrollment in a clinical trial should be considered for HCL-V. PMID: 21846972 [PubMed - as supplied by publisher] (Source: Acta Haematologica)

A Genetic Marker for Hairy Cell LeukemiaA Genetic Marker for Hairy Cell Leukemia

Medscape Today Headlines — Tue, 09 Aug 2011 11:04:29 +0100

This study reports on the presence of the BRAF V600E mutation in a cohort of hairy-cell leukemia patients, suggesting both a diagnostic marker and a potential pathogenetic mechanism. Journal Watch (Source: Medscape Today Headlines)

A New Therapeutic Target for Hairy Cell LeukemiaA New Therapeutic Target for Hairy Cell Leukemia

Medscape Today Headlines — Mon, 08 Aug 2011 15:16:00 +0100

Dr. Maurie Markman reviews a recent paper identifying a druggable BRAF mutation in patients with hairy cell leukemia. Medscape Genomic Medicine (Source: Medscape Today Headlines)

Mantle cell lymphoma: recent insights into pathogenesis, clinical variability, and new diagnostic markers

Seminars in Diagnostic Pathology — Tue, 12 Jul 2011 14:52:16 +0100

Mantle cell lymphoma (MCL; previously called centrocytic lymphoma or lymphocytic lymphoma of intermediate differentiation) is a distinct subtype of B-cell lymphoma, accounting for approximately 3%-10% of all lymphoma diagnoses. The name refers to the growth pattern in early disease presentation resembling the normal mantle zone that surrounds the germinal center of the B-cell follicle. The hallmark of MCL is the t(11;14)(q13;q32), resulting in aberrant expression of the CCND1 gene and expression of cyclin D1 in the tumor cells. Expression and genomic profiling of MCL have provided new insight into the pathogenesis and will be summarized in this review. Pitfalls in the differential diagnosis versus B-cell chronic lymphocytic leukemia, B-cell prolymphocytic leukemia, cyclin D1-positive diffu...

Comparative study between intravenous and subcutaneous administration of cladribine in treatment of hairy cell leukemia patients

Comparative Clinical Pathology — Wed, 06 Jul 2011 06:34:03 +0100

Abstract Cladribine is a cytotoxic agent with high activity in hematological malignancies. It is usually administered intravenously. The study was carried out on 20 Egyptian hairy cell leukemia cases; 17 cases were de novo, and 3 cases were relapsed. Four (20%) were treated with IV cladribine, and 16 (80%) were treated with subcutaneous (SC) cladribine. An average duration of response was 36 months with no relapses in cases receiving IV cladribine vs. 25% relapse rate in cases receiving SC cladribine, with no significant difference between them. The subcutaneous administration of cladribine has a positive benefit/risk ratio in the treatment of patients with hairy cell leukemia. Content Type Journal ArticlePages 1-6DOI 10.1007/s00580-011-1277-5Authors Nehad M. Ta...

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Histopathological audit of splenectomies received at a cancer hospital.

Indian Journal of Pathology and Microbiology — Fri, 01 Jul 2011 04:00:00 +0100

Conclusions: Predominantly splenic lymphoma is a biologically heterogeneous entity, ranging from low-grade SMZL to high-grade DLBCLs. TCLs constituted only 4% of all our cases. DRP, HCL, and HCLv have similar diffuse red pulp patterns of splenic involvement and are differentiated based on flow cytometric immunophenotyping. We had a large number of splenic DLBCL and none of these involved bone marrow (BM), while all other lymphoma subtypes had BM involvement (stage IV disease). Morphological and immunophenotypic (immunohistochemistry and flow cytometry) features of BM and splenectomy specimen need to be correlated to differentiate these rare though similar-looking entities with overlapping features. PMID: 21934208 [PubMed - in process] (Source: Indian Journal of Pathology and Microbiolo...

Gene Mutation Linked to Leukemia

Medical Headlines From Ivanhoe.com — Mon, 20 Jun 2011 05:00:00 +0100

(Ivanhoe Newswire) -- A new study reveals a single mutation, known as BRAF V600E, occurred in every patient in a group with hairy cell leukemia, suggesting this mutation could have implications for treating the disease. (Source: Medical Headlines From Ivanhoe.com)

Therapeutic potential of cladribine in combination with STAT3 inhibitor against multiple myeloma

BMC Cancer — Wed, 15 Jun 2011 23:00:00 +0100

Conclusions Cladribine exhibited inhibitory effects on MM cells in vitro. MM1.S is the only cell line showing significant response to the clinically achievable concentrations of cladribine-induced apoptosis and inactivation of STAT3. Our data suggest that MM patients with the features of MM1.S cells may particularly benefit from cladribine monotherapy, whereas cladribine in combination with STAT3 inhibitor exerts a broader therapeutic potential against MM. (Source: BMC Cancer)

BRAF Mutations in Hairy-Cell Leukemia

New England Journal of Medicine — Wed, 15 Jun 2011 21:00:08 +0100

New England Journal of Medicine, Volume 364, Issue 24, Page 2305-2315, June 2011. (Source: New England Journal of Medicine)

Hairy-Cell Leukemia Disease-Defining Mutation IdentifiedHairy-Cell Leukemia Disease-Defining Mutation Identified

Medscape Today Headlines — Mon, 13 Jun 2011 14:28:39 +0100

BRAF V600E has been identified as a disease-defining mutation, which opens up new avenues for diagnostic testing and drug targeting. Medscape Medical News (Source: Medscape Today Headlines)

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Single Mutation Key to Hairy Cell Leukemia (CME/CE)

MedPage Today Hematology/Oncology — Sat, 11 Jun 2011 13:00:00 +0100

(MedPage Today) -- A single mutation occurred in every patient in a cohort with hairy cell leukemia, suggesting that targeting the mutation could have major implications for treating the disease, Italian investigators reported. (Source: MedPage Today Hematology/Oncology)

Molecular Biologists Discover The Cause Of A Rare Type Of Leukemia

Health News from Medical News Today — Fri, 10 Jun 2011 15:00:00 +0100

During the annual meeting of the European Hematology Association (EHA) professor E. Tiacci from the Institute of Hematology in Perugia, Italy will present groundbreaking news on the cause of a specific, rare subtype of leukemia, so-called hairy cell leukemia. "With modern genetic technology we have discovered that in 46 out of 46 patients with hairy cell leukemia one specific gene in the DNA in the cell nucleus (BRAF gene) has undergone an irreversible change (called a "mutation")... (Source: Health News from Medical News Today)

Grey zone lymphoid neoplasms with features overlapping between splenic marginal zone lymphoma and hairy cell leukaemia: splenic B-cell lymphoma/leukaemia, unclassifiable

Journal of Hematopathology — Tue, 07 Jun 2011 15:51:24 +0100

Abstract The best characterized primary splenic B-cell lymphoma/leukaemias include splenic marginal zone lymphoma (SMZL) and hairy cell leukaemia (HCL). However, there are other primary splenic B-cell neoplasms that do not have the characteristic features of SMZL or HCL. The WHO classification has proposed a provision entity—splenic B-cell lymphoma/leukaemia, unclassifiable (SBLLu) to include the better-defined small B-cell clonal lymphoproliferations involving spleen that do not fall into any of the other types of B-cell lymphoid neoplasms. The recognized provisional entities among SBLLu include splenic diffuse red pulp small B-cell lymphoma (SDRPSBL) and hairy cell leukaemia variant (HCLv). The possibility of SBLLu arises when the spleen shows diffuse red pulp involvem...

Evidence of canonical somatic hypermutation in hairy cell leukemia

Blood — Wed, 04 May 2011 23:00:00 +0100

To compare hairy cell leukemia (HCL) with chronic lymphocytic leukemia (CLL) and normal B cells with respect to their B-cell receptors, somatic hypermutation (SHM) features in HCL were examined in a series of 130 immunoglobulin gene heavy chain rearrangements, including 102 from 100 classic (HCLc) and 28 from 26 variant (HCLv) patients. The frequency of unmutated rearrangements in HCLc was much lower than that in HCLv (17% vs 54%, P < .001) or historically in CLL (17% vs 46%, P < .001), but HCLv and CLL were similar (P = .45). As previously reported for CLL, evidence of canonical SHM was observed in HCLc rearrangements, including: (1) a higher ratio of replacement to silent mutations in the complementarity determining regions than in the framework regions (2.83 vs 1.41, P < .001),...

Hairy cell leukemia presenting as a cranial mass

American Journal of Hematology — Tue, 26 Apr 2011 15:08:33 +0100

(Source: American Journal of Hematology)

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Antitumour effects of single or combined monoclonal antibodies directed against membrane antigens expressed by human B cells leukaemia

Molecular Cancer — Mon, 18 Apr 2011 23:00:00 +0100

Conclusions: Altogether these data suggest that the combined use of two mAbs, such as anti-HLA-DR and anti-CD5, may significantly enhance their therapeutic potential. (Source: Molecular Cancer)

AAN: Case Report Links Cladribine to PML (CME/CE)

MedPage Today Hematology/Oncology — Wed, 13 Apr 2011 22:04:23 +0100

HONOLULU (MedPage Today) -- A patient treated with cladribine for hairy cell leukemia went on to develop progressive multifocal leukoencephalopathy, suggesting that the drug may pose a similar risk when used in multiple sclerosis, researchers reported here. (Source: MedPage Today Hematology/Oncology)

The expression of SOX11, cyclin D1, cyclin D2, and cyclin D3 in B-cell lymphocytic proliferative diseases

Medical Oncology — Sat, 09 Apr 2011 05:50:54 +0100

Abstract SOX11 is mainly correlated with embryo neurogenesis and remodeling of tissues. D cyclins (cyclin D1, cyclin D2, and cyclin D3) work in cell transformation. We assessed the expression of SOX11, cyclin D1, cyclin D2, and cyclin D3 mRNA in 152 patients with B-cell lymphocytic proliferative diseases (B-LPD) using qRT-PCR and we detected SOX11 protein using immunohistochemistry in 15 B-LPD patients, to clarify the clinical significance of the four genes in B-LPD. Data showed the transcriptional levels of SOX11 and cyclin D1 were higher for the mantle cell lymphoma (MCL) samples compared with chronic lymphocytic leukemia (CLL), diffuse large B-cell lymphoma (DLBCL), hairy cell leukemia (HCL), splenic marginal zone lymphoma (SMZL), and healthy collators. The expression l...

Recombinant immunotoxin against B-cell malignancies with no immunogenicity in mice by removal of B-cell epitopes [Medical Sciences]

Proceedings of the National Academy of Sciences — Mon, 04 Apr 2011 23:00:00 +0100

Many nonhuman proteins have useful pharmacological activities, but are infrequently effective in humans because of their high immunogenicity. A recombinant immunotoxin (HA22, CAT8015, moxetumomab pasudotox) composed of an anti-CD22 antibody variable fragment fused to PE38, a 38-kDa portion of Pseudomonas exotoxin A, has produced many complete remissions in drug-resistant hairy-cell leukemia when several cycles of the agent can be given, but has much less activity when antibodies develop. We have pursued a strategy to deimmunize recombinant immunotoxins by identifying and removing B-cell epitopes. We previously reported that we could eliminate most B-cell epitopes using a combination of point mutations and deletions. Here we show the location and amino acid composition of all of the B-cell ...

High-resolution genomic profiling in hairy cell leukemia-variant compared with typical hairy cell leukemia

Leukemia — Fri, 25 Mar 2011 00:00:00 +0100

Authors: S L Hockley, G J Morgan, P E Leone, B A Walker, A Morilla, M Else, A Wotherspoon, C Dearden, D Catovsky, D Gonzalez & E Matutes (Source: Leukemia)

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A case of coronary hypersensitivity (Kounis) syndrome associated with mid-ventricular ballooning pattern, intracoronary thrombosis and troponin elevation

International Journal of Cardiology — Mon, 21 Mar 2011 00:00:00 +0100

A 76-years old woman affected since 15years by a hairy cell leukemia underwent a treatment with rituximab. The patient had been previously treated with several chemotherapy and immunosuppressive regimens with inconstant results, and she had a history of multiple drug intolerance or allergy. During previous infusions of rituximab, she had presented mild, self-resolving, allergic reactions (cutaneous manifestations, mild dyspnea). During the current infusion of the monoclonal antibody, she reported more severe signs of allergic reaction, including chills, erythema, and dyspnea along with precordial pain. Although initially severe, the anaphylactic reaction regressed after administration of corticosteroids and histamine antagonists and the patient did not require treatment with adrenergic med...

Autoimmune Polyglandular Syndrome Type 2 Induced by Treatment With Interferon Alpha.

The American Journal of the Medical Sciences — Tue, 15 Mar 2011 00:00:00 +0100

Authors: Krysiak R, Boldys A, Okopien B Interferon α therapy has been reported to result in a variety of autoimmune side effects and to increase the risk of thyroid dysfunction. Autoimmune polyglandular syndromes are rarely described conditions characterized by the combination of at least 2 autoimmune endocrinopathies and nonendocrine autoimmunopathies, differing in the immunologic features of their pathogenesis. In light of research carried out in recent years, it seems that autoimmune polyendocrine syndromes occur much more frequently than previously estimated. In this article, the authors describe autoimmune polyglandular syndrome type 2 composed of autoimmune thyroid disease, Addison's disease and premature ovarian failure in a 37-year old woman after treatment of hairy cell leuke...

Nontuberculous mycobacterium genital infection mimicking donovanosis in a patient with hairy cell leukemia

Leukemia Research — Mon, 07 Mar 2011 00:00:00 +0100

Hairy cell leukemia (HCL) is an indolent and infrequent chronic B-cell lymphoproliferative disorder comprising 2–3% of all adult leukemias . It is characterized by the infiltration of the bone marrow and the spleen by mature lymphocytes with typical cytoplasmic projections (hairy cells). These cells strongly express CD20, CD22 and monoclonal surface immunoglobulins, together with the distinctive expression of CD25, CD11c, FMC7 and CD103 . (Source: Leukemia Research)

Immune mechanisms of new therapeutic strategies in MS - Teriflunomide.

Clinical Immunology — Mon, 28 Feb 2011 00:00:00 +0100

Authors: Claussen MC, Korn T At present, a series of oral disease-modifying agents is being introduced for the treatment of multiple sclerosis. With the exception of laquinimod, the "new" oral compounds have already been approved for other indications such as organ transplantation (FTY720), psoriasis (dimethylfumarate), hairy cell leukemia (cladribine), and rheumatoid arthritis (leflunomide). Leflunomide is the prodrug of teriflunomide which is the latest compound that has successfully been tested in a large phase III clinical trial in relapsing MS. Due to its favorable safety profile and its efficacy in rheumatoid arthritis where the aberrant immune response is in various aspects similar to the autoimmune reaction in MS patients, teriflunomide is a promising treatment option for MS pa...

Hairy cell leukemia and variant in Taiwan: report of a variant case and literature review.

International Journal of Clinical and Experimental Pathology — Sun, 20 Feb 2011 16:45:03 +0100

We present the case of a 67-year-old Taiwanese male with HCL-v who had leukocytosis and splenomegaly. His hairy leukemic cells were weakly positive for TRAP and expressed CDllc and CD103 but not CD25. He received oral chemotherapy with chlorambucil and in complete hematological remission in 9 months but relapsed 2 months later. Literature review revealed 9 cases of HCL and 3 cases of HCL-v including current case from Taiwan. All patients were adults with splenomegaly. The HCL patients had a significantly higher frequency of leukopenia (p = 0.024) and monocytopenia (p = 0.008) and a lower frequency of leukocytosis (p = 0.018) than HCL-v patients. All 8 HCL patients responded favorably to 2-chlorodeoxyadenosine with or without splenectomy. The 3 HCL-v patients had leukocytosis and received c...

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Modern Strategies for Hairy Cell Leukemia [REVIEW ARTICLES]

Journal of Clinical Oncology — Tue, 08 Feb 2011 00:00:00 +0100

Enormous progress in the treatment of hairy cell leukemia over the last five decades has emerged as a result of organized clinical investigations. Although interferon represented one of the initial major therapeutic advances in the management of this disease in 1984, the subsequent introduction of purine nucleoside analogs (pentostatin and cladribine) changed the natural history of this rare disease by achieving a high rate of complete and durable remissions. The disease-free survival after effective therapy has not reached a plateau, suggesting control but not cure of the disease. Identification of minimal residual disease in patients achieving a complete hematologic remission provides insight into the potential source for predicting eventual relapse. Modern strategies of targeted therapi...

Review: Modern strategies for hairy cell leukaemia

NeLM - News — Tue, 11 Jan 2011 00:00:00 +0100

Source: JCO Area: News In this review on hairy cell leukaemia, the following topics are discussed: . Enormous progress through clinical investigation . Establishing the correct diagnosis and initiating therapy . Management decisions . Importance of minimal residual disease . Relapse and unresponsive disease . Potential new avenues for therapeutic research (Source: NeLM - News)

CD20dim-positive T-cell large granular lymphocytic leukemia in a patient with concurrent hairy cell leukemia and plasma cell myeloma.

International Journal of Clinical and Experimental Pathology — Sun, 26 Dec 2010 11:20:02 +0100

We report a CD20dim- positive T-cell large granular lymphocytic (T-LGL) leukemia in a patient with concurrent hairy cell leukemia and plasma cell myeloma. This patient was first diagnosed with T-LGL leukemia with dim CD20 expression, which by itself was a rare entity. He received no treatment for T-LGL leukemia. The patient later developed a hairy cell leukemia, which went into complete clinical remission after one cycle of 2-CdA. Five years later, he was diagnosed with a third malignancy, plasma cell myeloma. Complex cytogenetic aberrancies were present at the time when plasma cell myeloma was diagnosed. This is the first report, to the best of our knowledge, in the English literature with the aforementioned three distinct hematopoietic malignancies in one patient. PMID: 21151394 [Pub...

Splenic B-Cell Lymphomas/Leukemias

Surgical Pathology Clinics — Wed, 01 Dec 2010 00:00:00 +0100

This article discusses the diagnostic approach to splenic-based lymphoproliferative disorders in routine practice and describes the clinicopathologic features of lymphoid neoplasms that characteristically present in the spleen. (Source: Surgical Pathology Clinics)

Hairy cell leukemia variant

Journal of Hematopathology — Thu, 18 Nov 2010 17:57:14 +0100

In this report we aim to add a description of the splenic histology, and extend our knowledge of HCLv. We describe one case of this entity for which a splenic histology was available. It fulfilled all the diagnostic criteria for HCLv, exhibiting a characteristic pattern of splenic infiltration, and responded favorably to treatment. The case described seems to fit well with HCLv where neither morphology nor immunophenotype bears any relation with hairy cell leukemia. Content Type Journal ArticleDOI 10.1007/s12308-010-0077-1Authors George Kanellis, Haematopathology Department, Evangelismos Hospital, Perikleus 11, 17343 Athens, GreeceLuis Garcia-Alonso, Department of Haematology, Hospital Universitario de Getafe, Madrid, SpainFrancisca I. Camacho, Department of Pathology, Hospital ...

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Hairy cell leukaemia presenting as a cranial mass

American Journal of Hematology — Mon, 15 Nov 2010 00:00:00 +0100

AbstractA 42 year old gentleman presented with a one month history of occipital swelling, on a background of intermittent headaches over the previous six months. He was otherwise fit and well. He had suffered a head injury two years earlier but there was no other past medical history of note and he took no regular medications. His family history revealed a sibling and an aunt to have had Hodgkin's lymphoma. He was a non‐smoker and drank 21 units of alcohol per week. Examination confirmed a right sided occipital swelling but was otherwise unremarkable. Am. J. Hematol., 2010. © 2010 Wiley‐Liss, Inc. (Source: American Journal of Hematology)

Molecular and flow cytometry characterization during the follow‐up of three simultaneous lymphoproliferative disorders: Hairy cell leukemia, monoclonal B‐cell lymphocytosis, and CD4++/CD8+/−dim T‐large granular lymphocytosis—A case report

Cytometry Part B: Clinical Cytometry — Mon, 01 Nov 2010 00:00:00 +0100

We report the simultaneous presence of hairy cell leukemia, monoclonal B‐cell lymphocytosis, and alpha beta CD4++/CD8+ T‐cell large granular lymphocytosis in a 63‐year‐old man. After the diagnosis, the three lymphoproliferative disorders (i.e., two of B‐cell lineage and one of T‐cell lineage) were characterized by analysis of multiple sequential bone marrow and peripheral blood samples using flow cytometry and molecular techniques. We discuss these findings in the context of chronic antigen stimulation, immunosuppression, and apoptotic pathway alterations, which might be implicated in the accumulation of these abnormal clones in the same patient. Because the phenotype of the three clones is compatible with fully differentiated B lymphocytes (consistent with a postgerminal origi...

CD200 (OX-2 Membrane Glycoprotein) Expression in B Cell-Derived Neoplasms.

American Journal of Clinical Pathology — Mon, 25 Oct 2010 22:21:20 +0100

Authors: Dorfman DM, Shahsafaei A We studied the expression of CD200, an immunoglobulin superfamily membrane glycoprotein, in a wide range of B cell-derived neoplasms by immunohistochemical staining of paraffin-embedded tissue sections. In addition to chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL), CD200 is expressed in other B-cell lymphoproliferative disorders, including hairy cell leukemia. In addition, neoplastic cells in classical Hodgkin lymphoma are immunoreactive for CD200. CD200 was previously reported to be expressed in acute myeloid leukemia, and we find that it is also expressed in B-lymphoblastic leukemia/lymphoma. We conclude that CD200 may be a useful immunophenotypic marker in the evaluation of B cell-derived neoplasms. Furthermore, since an anti-CD...

Beyond hairy cell: the activity of cladribine in other hematologic malignancies

Blood — Wed, 20 Oct 2010 23:00:00 +0100

Before the contemporary development of rationally designed antineoplastic therapies, cladribine was identified as a lymphocyte-specific agent. Its profound impact on the natural history of hairy cell leukemia, with responses approaching 100% and a median duration of response of nearly a decade after only a single 7-day course, is well known and revolutionized the treatment of hairy cell leukemia. However, cladribine's impressive activity in other lymphoproliferative disorders has been generally underappreciated. Multiple single-arm phase 2 trials have demonstrated cladribine's potency across the full spectrum of lymphoid malignancies. In a limited number of phase 3 trials and cross-study analyses, cladribine compared favorably with fludarabine, another purine nucleoside analog that is more...

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Autoimmune conditions and hairy cell leukemia: an exploratory case-control study

Journal of Hematology and Oncology — Sun, 03 Oct 2010 23:00:00 +0100

Background: Case reports suggest that hairy cell leukemia (HCL) may be associated with autoimmune conditions, however no systematic investigations in this area have been undertaken. Methods: Using the United States Surveillance, Epidemiology, and End Results Medicare linked database, we conducted an exploratory study comparing autoimmune conditions in 418 HCL cases (aged [greater than or equal to]65 years) and 160,086 controls. Results: Overall, the proportion with autoimmune conditions was similar between HCL cases and controls (n=79 (18.9%) and n=29,284 (18.3%), respectively). Before diagnosis/selection, there was no overall difference in the prevalence of autoimmune conditions in HCL cases (n=37, 8.9%) compared with controls (n=14,085, 8.8%), p=0.969. However, compared with controls, HC...

Characterisation of 7q32 deletion of splenic marginal zone lymphoma demonstrates frequent miR-182 somatic mutations in a wide range of lymphoma subtypes.

Haematologica — Thu, 16 Sep 2010 23:00:00 +0100

Authors: Watkins AJ, Hamoudi R, Liu H, Zhang J, de Leval L, Isaacson P, Wotherspoon A, Du MQ Splenic marginal zone lymphoma is characterised by 7q32 deletion, but the target genes of the deletion remain unknown. The minimally deleted region contains a cluster of 6 microRNAs. We investigated whether these miRNAs are the target of 7q32 deletion. High resolution array comparative genomic hybridisation showed no evidence of cryptic or homozygous deletions of the microRNAs. Quantitative polymerase chain reaction showed reduced expression of the miRNA cluster in splenic marginal zone lymphoma relative to other lymphomas, with cases bearing the 7q deletion showing downregulation compared to those without the deletion. Sequencing of these microRNAs revealed a single recurrent somatic mutation ...

Isolated Splenic Mycobacterial Disease: A Cause of Persistent Fever in a Hairy Cell Leukemia Patient

Karger Publishers — Fri, 10 Sep 2010 23:00:00 +0100

Case Rep Gastroenterol 2010;4:330â334 (DOI:10.1159/000320639) (Source: Karger Publishers)

Hairy cell leukaemia: biological and clinical overview from immunogenetic insights

Hematological Oncology — Wed, 01 Sep 2010 00:00:00 +0100

AbstractHairy cell Leukaemia (HCL) is a rare neoplasm of peripheral B cells which represents a paradox in oncology. Despite its largely unknown origin and behaviour, HCL is one of the few example of dramatic success in the treatment of a malignancy. The recent steps forward to understanding the biology of HCL from immunogenetic and genomic studies have recently provided new insight into diagnosis and prognosis. Several data from immunoglobulin gene (IG) analysis have provided hints regarding the cell of origin and the ongoing selective interactions of the tumour BCR with environmental stimuli. It has also recently emerged that an unmutated status of the HCL IG can be associated with failure to respond to cladribine, genetic abnormalities indicative of poor outcome and aggressive disease. T...

Variables in the quantification of CD4 in normals and Hairy cell leukemia patients

Cytometry Part B: Clinical Cytometry — Tue, 03 Aug 2010 23:00:00 +0100

Quantitative flow cytometry (QFCM) is being applied in the clinical flow cytometry laboratory. Quantitative normal T-cell CD4 expression represents a biologic standard and quality control agent. However, low levels of CD4 expression were detected in normal T-cells in Hairy Cell Leukemia (HCL) samples.The QuantiBrite System® was used to determine the level of CD4 expression (mean antibody bound per cell, ABC) in fresh and shipped HCL blood and fresh normal donor blood (NDB). The effects of shipping, lysing reagent, cell preparation method, and antibody lot were evaluated.Shipped HCL specimens (n = 69) had a significantly lower mean CD4 ABC of 38,788 (CV = 9.1%) compared to fresh specimens (n = 105) CD4 value of 40,330 (CV = 8.4%) (P < 0.05). In NDB, significant differences were seen for fr...

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Sustained long‐term remissions with weekly interferon maintenance therapy in hairy cell leukemia

Asia-Pacific Journal of Clinical Oncology — Sat, 31 Jul 2010 23:00:00 +0100

(Source: Asia-Pacific Journal of Clinical Oncology)

images in haematology: Hairy cell leukaemia, negative for conventional cell markers, diagnosed using antibodies to Annexin A1 and T‐bet

British Journal of Haematology — Sat, 31 Jul 2010 23:00:00 +0100

(Source: British Journal of Haematology)

Lack of allelic exclusion by secondary rearrangements of tumour B-cell receptor light chains in hairy cell leukaemia

Hematological Oncology — Fri, 23 Jul 2010 23:00:00 +0100

Analyses of the tumour immunoglobulin (Ig) gene (IG) heavy (H) and light chains show heterogeneity of mutational status, but reveal common features of ongoing IGH isotype-switching with multiple IGH isotype expression and preference of IG lambda (IGL) light chain with selective use of IGLJ3. Phenotypic and immunogenetic analyses were performed in a series of 105 HCL patients to estimate prevalence of multiple IG light chain expression by the tumour cells. By phenotype, 3/105 HCL (2.9%) expressed double tumour-related Ig kappa (K) and L light chain proteins. By immunogenetic analysis, functional mutated double IGKI/IGKII, IGKI/IGLI and IGLI/IGLII transcripts were cloned and sequenced in 3/71 (4.2%) HCL. These latter three HCL expressed multiple IGH isotypes with mutated IGHVDJ rearrangement...

Irreversible marrow aplasia after single course of 2-chlorodeoxyadenosine for hairy cell leukaemia preceding by A pandemic 2009-H1N1-associated pneumonia

Medical Oncology — Fri, 23 Jul 2010 12:09:41 +0100

We report a HCL patient with A pandemic 2009-H1N1-associated pneumonia who fully recovered after oseltamivir and antibiotics. The subsequent treatment with single 5-day course of 2-CdA resulted in persistent marrow aplasia with fatal systemic aspergillosis. Content Type Journal ArticleCategory Original paperDOI 10.1007/s12032-010-9626-9Authors Grzegorz Helbig, Silesian Medical University Department of Haematology and Bone Marrow Transplantation Dabrowski Street 25 40-032 Katowice PolandKrzysztof Woźniczka, Silesian Medical University Department of Haematology and Bone Marrow Transplantation Dabrowski Street 25 40-032 Katowice PolandAgata Wieczorkiewicz, Silesian Medical University Department of Haematology and Bone Marrow Transplantation Dabrowski Street 25 40-032 Katowice Poland...

Sustained long-term remissions with weekly interferon maintenance therapy in hairy cell leukemia

Asia-Pacific Journal of Clinical Oncology — Tue, 20 Jul 2010 23:00:00 +0100

Conclusion: Our results are similar to those from three previous studies using long-term IFN maintenance therapy, bringing the total number of patients in sustained remission to 118. We hope these reports will lead to a multi-centre, phase III study of IFN maintenance therapy (including pegylated IFN, given less frequently) in HCL patients achieving optimal response to initial therapy, be it IFN or a purine analogue. (Source: Asia-Pacific Journal of Clinical Oncology)

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Hairy cell leukemia

Cancer — Wed, 30 Jun 2010 23:00:00 +0100

Therapy of B-cell malignancies by anti-HLA-DR humanized monoclonal antibody, IMMU-114, is mediated through hyperactivation of ERK and JNK MAP kinase signaling pathways

Blood — Thu, 24 Jun 2010 16:01:57 +0100

A humanized IgG4 anti–HLA-DR monoclonal antibody (IMMU-114), engineered to avoid side effects associated with complement activation, was examined for binding and cytotoxicity on leukemia, lymphoma, and multiple myeloma cell lines and chronic lymphocytic leukemia (CLL) patient specimens, followed by evaluation of the effects of IMMU-114 on extracellular signal-regulated kinase (ERK) and c-Jun N-terminal kinase (JNK) signaling pathways. HLA-DR was expressed on the majority of these cells at markedly higher levels than CD20, CD22, and CD74. IMMU-114 was toxic to mantle cell lymphoma, CLL, acute lymphoblastic leukemia, hairy cell leukemia, non-Hodgkin lymphoma (including rituximab-resistant), and multiple myeloma cell lines, and also patient CLL cells. IMMU-114 induced disease-free survi...

Hairy-cell leukemia variant: Recent view on diagnosis, biology and treatment

Cancer Treatment Reviews — Thu, 17 Jun 2010 00:00:00 +0100

Abstract: Hairy-cell leukemia variant (HCl-V) is a district clinico-pathological entity with intermediate features between classical HCl (HCl-C) and B-cell prolymphocytic leukemia. HCl-V is now included in the World Health Organization (WHO) classification as a provisional entity. It is an uncommon disorder accounting for approximately 0.4% of chronic lymphoid malignancies and 10% of all HCl cases. In contrast to HCl-C, HCl-V is a more aggressive disease and according to the new WHO classification it is no longer considered to be biologically related to HCl-C. Patients with HCl-V have an elevated white blood count, easy-to-aspirate bone marrow and weak reactivity to tartrate – resistant acid phosphatase (TRAP). Immunophenotypically, HCl-V cells are positive for CD103 and CD11c and negati...

In vivo targeting of B-cell lymphoma with glycan ligands of CD22

Blood — Thu, 10 Jun 2010 16:03:34 +0100

Antibody-mediated cell depletion therapy has proven to provide significant clinical benefit in treatment of lymphomas and leukemias, driving the development of improved therapies with novel mechanisms of cell killing. A current clinical target for B-cell lymphoma is CD22, a B-cell–specific member of the sialic acid binding Ig-like lectin (siglec) family that recognizes 2-6–linked sialylated glycans as ligands. Here, we describe a novel approach for targeting B lymphoma cells with doxorubicin-loaded liposomal nanoparticles displaying high-affinity glycan ligands of CD22. The targeted liposomes are actively bound and endocytosed by CD22 on B cells, and significantly extend life in a xenograft model of human B-cell lymphoma. Moreover, they bind and kill malignant B cells from peri...

Interferon-alpha is a very effective salvage therapy for patients with hairy cell leukemia relapsing after cladribine: a report of three cases

Medical Oncology — Fri, 28 May 2010 15:13:00 +0100

Abstract Therapy with a purine analogue (cladribine or pentostatin, most often cladribine) has revolutionized the care of patients with hairy cell leukemia, resulting in very long-lasting remissions in the majority of cases. For patients who relapse, re-induction with cladribine again induces durable remissions. For patients with short remission durations, rituximab can be effective, and for a small proportion of patients with more resistant disease, BL22 has been used successfully. I report three cases in which alpha interferon induction and maintenance has produced ongoing remissions in patients with short remission durations after cladribine. One patient had also not responded to rituximab. Alpha interferon may be a very effective treatment option for selected patients ...

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A paraneoplastic membranoproliferative glomerulonephritis with isolated C3 deposits associated with hairy cell leukaemia

Nephrology Dialysis Transplantation — Tue, 25 May 2010 16:35:08 +0100

We describe a 35-year-old woman who presented with proteinuria and microscopic haematuria. Blood tests revealed a low C3 complement level, with no evidence of cryoglobulin. Renal biopsy showed a Type 1 membranoproliferative glomerulonephritis (MPGN) with isolated C3 deposits on immunofluorescence study. Bone marrow aspirate, done for monocytopenia, was consistent with a diagnosis of hairy cell leukaemia (HCL). Both haematological and nephrological diseases completely responded to treatment with cladribine, strongly suggesting that the renal disease was a paraneoplastic syndrome. To our knowledge, this is the first report of a non-cryoglobulinaemic MPGN associated to HCL. (Source: Nephrology Dialysis Transplantation)

Immunoglobulin a nephropathy associated with hairy cell leukaemia: a case report

Nephrology — Mon, 24 May 2010 23:00:00 +0100

(Source: Nephrology)

Diffuse Osteosclerosis Complicating Hairy Cell Leukemia [DIAGNOSIS IN ONCOLOGY]

Journal of Clinical Oncology — Thu, 29 Apr 2010 22:02:09 +0100

(Source: Journal of Clinical Oncology)

EUS-guided biopsy for the diagnosis and classification of lymphoma

Gastrointestinal Endoscopy — Wed, 31 Mar 2010 23:00:00 +0100

Conclusion: EUS-guided biopsy has a lower yield to correctly classify Hodgkin lymphoma and low-grade lymphoma compared with high-grade diffuse large B-cell lymphoma. (Source: Gastrointestinal Endoscopy)

Mycotic pulmonary artery aneurysm due to Aspergillus infection in a patient with leukemia: Case report and review of the literature

Leukemia Research — Tue, 30 Mar 2010 13:31:14 +0100

We present a case of a patient with hairy cell leukemia and pulmonary aspergillosis who developed a cycotic pulmonary artery aneurysm despite prolonged antifungal therapy. A review of the literature in regards to incidence, etiology, clinical manifestations and treatment options is included. (Source: Leukemia Research)

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Lymphoma – Emerging realities in sub-Saharan Africa

Transfusion and Apheresis Science — Tue, 23 Mar 2010 14:35:44 +0100

Abstract: Substantial geographical differences exist for Hodgkin and other lymphoproliferative disorders with these having previously been documented in a report from the lymphoma reclassification project. In the light of rampant human immunodeficiency syndrome, largely centred in sub-Sahara, this experience is updated in a further 512 consecutive individuals treated over an 8-year period in a privately based academic centre.Median age was 55.2years 61% were males, 10% had Hodgkin lymphoma and, overall, constitutional symptoms were present in 20%. Prior to referral 19% had received chemotherapy and a further 20% some form of irradiation.Median survival in hairy cell leukaemia (n=14), chronic lymphocytic leukaemia–small lymphocytic lymphoma (n=103), Hodgkin (n=41) and follicular lymphoma ...

Higher expression levels of activation-induced cytidine deaminase distinguish hairy cell leukemia from hairy cell leukemia-variant and splenic marginal zone lymphoma

Leukemia — Thu, 18 Mar 2010 00:00:00 +0100

Authors: S L Hockley, A Morilla, M Else, C Dearden, D Catovsky, G J Morgan, E Matutes & D Gonzalez (Source: Leukemia)

Hairy cell leukaemia-variant and splenic red pulp lymphoma: a single entity?

British Journal of Haematology — Fri, 12 Mar 2010 00:00:00 +0100

(Source: British Journal of Haematology)

Very long-term eradication of minimal residual disease in patients with hairy cell leukemia after a single course of cladribine

Blood — Thu, 11 Mar 2010 17:02:02 +0100

Cladribine induces protracted remissions in patients with hairy cell leukemia (HCL). However, many long-term responders ultimately relapse. We sought to determine whether long-term complete responders subsequent to a single 7-day course of cladribine were without minimal residual disease (MRD) and potentially cured of HCL. From the 358-person Scripps Clinic cladribine database, we identified 19 patients in continuous and complete hematologic response (median age, 75 years; median time from diagnosis, 18 years; and median time from cladribine, 16 years). Nine of 19 (47%) patient samples had no evidence of residual disease; 7 of 19 (37%) samples had MRD; and 3 of 19 (16%) had morphologic evidence of HCL in hematoxylin and eosin–stained bone marrow sections. These results indicate that ...

Identification of uniquely expressed transcription factors in highly purified B-cell lymphoma samples

American Journal of Hematology — Tue, 09 Mar 2010 00:00:00 +0100

In this study, global GEP analyses were performed on highly purified, flow-cytometry sorted tumor-cells from eight subgroups of BCLs. This enabled identification of TFs that can be uniquely associated to the tumor cells of chronic lymphocytic leukemia (CLL), diffuse large B-cell lymphoma (DLBCL), follicular lymphoma (FL), hairy cell leukemia (HCL), and mantle cell lymphoma (MCL). The identified transcription factors influence both the global and specific gene expression of the BCLs and have possible implications for diagnosis and treatment. Am. J. Hematol., 2010. © 2010 Wiley-Liss, Inc. (Source: American Journal of Hematology)

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Lymphomas involving the spleen

Diagnostic Histopathology — Mon, 01 Mar 2010 00:00:00 +0100

Abstract: Splenectomy is undertaken for diagnosis, and in later stages of management, of patients with a diverse range of lymphomas. High quality histological sections, requiring careful attention to tissue fixation, are needed to assess these lesions adequately. Increasing use of fine-needle aspiration, needle core biopsy and laparoscopic surgery add further diagnostic challenges. In addition to involvement by dissemination of lymphomas based primarily in lymph nodes, bone marrow or other tissues, spleen is the predominant site of disease in several distinctive types of lymphoma. In particular, splenic marginal zone B-cell lymphoma, hairy cell leukaemia and T-cell and macrophage-rich large B-cell lymphoma are recognized as clinicopathologically distinct entities. Research into the cellula...

Cladribine led to long-term eradication of hairy cell leukemia

HemOncToday.com — Mon, 15 Feb 2010 15:20:00 +0100

(Source: HemOncToday.com)

Two cases of disseminated Mycobacterium avium infection associated with a new immunodeficiency syndrome related to CXCR4 dysfunctions

Clinical Microbiology and Infection — Tue, 09 Feb 2010 00:00:00 +0100

We report two cases of disseminated MAC disease in young women with extended granulomatosis that revealed a new form of severe immunodeficiency syndrome. Both clinical observations initially appeared to be very similar to WHIM syndrome (Warts, Hypogammaglobulinemia, Infection, Myelokathexis), a rare immunodeficiency disease correlated with CXC chemokine receptor 4 (CXCR4) mutation leading to an impaired internalization of the receptor upon its ligand CXCL12. We investigated the CXCR4 status of the lymphocytes in both patients and found a severe defect in CXCL12-promoted internalization but no mutation of its gene. Moreover, myelokathexis was not noted in bone marrow biopsies and therefore a diagnosis of WHIM syndrome could not be assessed. This immunodeficiency syndrome associated with CXC...

How I treat hairy cell leukemia

Blood — Thu, 07 Jan 2010 17:02:10 +0100

The description of hairy cell leukemia as a specific clinical entity was published 50 years ago. The clinical outcome for patients was hampered by ineffective chemotherapy, and splenectomy was the major therapeutic approach to improve peripheral blood counts. The median survival after diagnosis was 4 years. With the introduction of -interferon in 1984, marked improvements in patient responses were observed. Shortly thereafter, the introduction of the purine nucleoside analogs transformed this disease into a highly treatable form of leukemia, and patients with the classic form of this rare leukemia now have a near-normal life expectancy. However, other clinical entities mimicking this disease do not respond; thus, accurate diagnosis is important. Immunophenotypic features in classic hairy c...

Primary autoimmune myelofibrosis in a 36-year-old patient presenting with isolated extreme anemia

Leukemia Research — Fri, 01 Jan 2010 00:00:00 +0100

Fibrosis of the bone marrow is a pathological condition associated with a variety of bone marrow neoplasms, including, for example, myeloproliferative neoplasms (MPN), acute megakaryoblastic leukemia, and hairy cell leukemia. Bone marrow fibrosis can also be seen with infections of the bone marrow, such as tuberculosis, or associated with metastatic disease in the bone marrow from solid tumors . In cases where a cause for bone marrow fibrosis cannot be readily identified, primary myelofibrosis (PMF) is frequently diagnosed. Histological features of bone marrow in PMF include significant deposits of extracellular matrix proteins, increased angiogenesis, osteosclerosis and increased hematopoiesis . Patients with PMF usually present with anemia, peripheral blood smear showing teardrop red cel...

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Lack of allelic exclusion by secondary rearrangements of tumour B‐cell receptor light chains in hairy cell leukaemia

Hematological Oncology — Fri, 01 Jan 2010 00:00:00 +0100

(Source: Hematological Oncology)

Which Role for Rituximab in Hairy Cell Leukemia? Reflections on Six Cases.

Acta Haematologica — Thu, 24 Dec 2009 00:00:00 +0100

Authors: Malfuson JV, Fagot T, Konopacki J, Souleau B, Cremades S, de Revel T Hairy cell leukemia (HCL) is a rare, chronic, B-cell, lymphoproliferative disorder. Treatment has been revolutionized by the advent of interferon (IFN)-alpha and purine analogs (PA). First-line therapy with PA yields complete response rates of 75-100%, with many long-lasting remissions. In the event of profound neutropenia and/or infectious complications, a short sequence of IFN-alpha may precede PA treatment. Because of the excellent results achieved with PA therapy, the potential role of rituximab (an anti-CD20 monoclonal antibody that is highly effective against most B-cell lymphomas) in HCL has yet to be elucidated. Six HCL cases treated with rituximab are reported herein with a view to elucidating the po...

Which Role for Rituximab in Hairy Cell Leukemia? Reflections on Six Cases

Karger Publishers — Wed, 23 Dec 2009 23:00:00 +0100

Acta Haematol 2010;123:110-116 (DOI:10.1159/000270903) (Source: Karger Publishers)

Splenic marginal zone lymphoma: characterization of 7q deletion and its value in diagnosis

The Journal of Pathology — Mon, 30 Nov 2009 00:00:00 +0100

In conclusion, 7q32 deletion is a characteristic feature of SMZL, albeit seen in isolated cases of splenic B-cell lymphoma/leukaemia unclassifiable, and its detection may help the differential diagnosis of splenic B-cell lymphomas. Copyright © 2009 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd. (Source: The Journal of Pathology)

Initial characterization of an immunotoxin constructed from domains II and III of cholera exotoxin

Cancer Immunology, Immunotherapy — Sun, 29 Nov 2009 09:38:18 +0100

Abstract Immunotoxins are antibody–toxin fusion proteins under development as cancer therapeutics. In early clinical trials, immunotoxins constructed with domains II and III of Pseudomonas exotoxin (termed PE38), have produced a high rate of complete remissions in Hairy Cell Leukemia and objective responses in other malignancies. Cholera exotoxin (also known as cholix toxin) has a very similar three-dimensional structure to Pseudomonas exotoxin (PE) and when domains II and III of each are compared at the primary sequence level, they are 36% identical and 50% similar. Here we report on the construction and activity of an immunotoxin made with domains II and III of cholera exotoxin (here termed CET40). In cell viability assays, the CET40 immunotoxin was equipotent to tenfo...

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Yield of Bone Marrow Examination in Diagnosing the Source of Fever of Unknown Origin [Original Investigation]

Archives of Internal Medicine — Mon, 23 Nov 2009 20:51:32 +0100

Conclusions Bone marrow biopsy is a useful technique for the diagnosis of prolonged fever in immunocompetent patients. Thrombocytopenia and anemia seem to be correlated with the value of this test. (Source: Archives of Internal Medicine)

VH4-34+ hairy cell leukemia, a new variant with poor prognosis despite standard therapy

Blood — Thu, 19 Nov 2009 17:02:20 +0100

Hairy cell leukemia variant (HCLv) presents with high disease burden, lack of typical antigens like CD25, and poor response to standard treatments like cladribine. Occasionally, patients with classic HCL respond poorly. Clinical and molecular features of HCL and HCLv has not been compared. Rearrangements expressing immunoglobulin VH chain were sequenced, including 22 from 20 patients with HCLv and 63 from 62 patients with classic HCL. Most patients were seeking relapsed/refractory trials, representing a poor-prognosis population. VH4-34, a gene commonly used in autoimmune disorders, was observed in 8 (40%) HCLv and 6 (10%) classic (P = .004) HCL patients. Compared with 71 VH4-34– rearrangements, 14 VH4-34+ rearrangements were more frequently (P < .001) unmutated, defined as greate...

Hairy cell leukemias with unmutated IGHV genes define the minor subset refractory to single-agent cladribine and with more aggressive behavior

Blood — Thu, 19 Nov 2009 17:02:20 +0100

Hairy cell leukemia (HCL) is generally responsive to single-agent cladribine, and only a minority of patients are refractory and with poor prognosis. HCLs generally express mutated (M) and, in a minority, unmutated (UM) IGHV. In a multicenter clinical trial in newly diagnosed HCL, we prospectively investigated clinical and molecular parameters predicting response and event-free survival after single-agent cladribine. Of 58 HCLs, 6 expressed UM-IGHV (UM-HCL) and 52 M-IGHV (M-HCL). Beneficial responses were obtained in 53 of 58 patients (91%), whereas treatment failures were observed in 5 of 58 patients (9%). Failures were associated significantly with UM-IGHV (5 of 5 failures vs 1 of 53 beneficial responses had UM-IGHV, P < .001), leukocytosis (3 of 5 vs 3 of 53, P = .006), and bulky spl...

IG genes and hairy cell leukemia

Blood — Thu, 19 Nov 2009 17:02:19 +0100

(Source: Blood)

Persistent non-neoplastic gammadelta-T cells in cerebrospinal fluid of a patient with hepatosplenic (gammadelta) T cell lymphoma: a case report with 6 years of flow cytometry follow-up.

International Journal of Clinical and Experimental Pathology — Thu, 19 Nov 2009 10:38:02 +0100

We describe a case of a 31-year old man diagnosed with a gammadelta HSTCL in 2003, successfully treated with chemotherapy and allogeneic stem cell transplantation, and followed from 2003 to present. Four-color flow cytometry (FC) was performed on a BD FACSCalibur and data analyzed with CellQuest Pro and FCS Express software. For cerebrospinal fluid (CSF), all cells were acquired due to limited material. Cytological correlation was available on all specimens. Molecular studies for T-cell gene rearrangement were non-contributory. By FC, the diagnostic HSTCL immunophenotype was CD3 (+), CD7 (+), CD2 (+), CD5 (-), CD4 (-), CD8 (-), TCR gammadelta (+). Subsequent CSF FC analysis revealed a distinct population of gammadelta T-cells in all specimens, ranging from <1% to 13% of lymphocytes. Con...

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Biology and Clinical Significance of Tartrate-Resistant Acid Phosphatases: New Perspectives on an Old Enzyme

Calcified Tissue International — Sat, 14 Nov 2009 06:58:49 +0100

Abstract Type 5 tartrate-resistant acid phosphatase (TRAP) has been a clinically relevant biomarker for about 50 years. It has always been a reliable and specific cytochemical marker for hairy cell leukemia and for differentiated cells of monocytic lineage. Only recently has the test for serum TRAP activity been accepted as sensitive and specific enough for clinical use as a marker of osteoclasts and bone resorption. This has come about through steady advances in knowledge about TRAP enzymology, structure, function, and molecular regulation and a consequent appreciation that TRAP isoforms 5a and 5b have very different clinical significance. As a measure of osteoclast number and bone resorption, TRAP 5b has diagnostic and prognostic applications in osteoporosis, cancer...

Strong lymphoid nuclear expression of SOX11 transcription factor defines lymphoblastic neoplasms, mantle cell lymphoma and Burkitt's lymphoma.

Haematologica — Sun, 01 Nov 2009 00:00:00 +0100

Conclusions In addition to mantle cell lymphoma, SOX11 is strongly expressed only in lymphoblastic malignancies and Burkitt's lymphomas. Its expression is independent of cyclin D1 (except for weak expression in hairy cell leukemias) and unlikely to be due to translocations in lymphoid neoplasia. PMID: 19880779 [PubMed - in process] (Source: Haematologica)

Insight into the molecular pathogenesis of hairy cell leukaemia, hairy cell leukaemia variant and splenic marginal zone lymphoma, provided by the analysis of their IGH rearrangements and somatic hypermutation patterns

British Journal of Haematology — Thu, 22 Oct 2009 23:00:00 +0100

(Source: British Journal of Haematology)

Hairy cell leukemia responsive to anti-thymocyte globulin used as immunosuppressive therapy for aplastic anemia.

International Journal of Hematology — Tue, 13 Oct 2009 23:00:00 +0100

Authors: Fujiwara S, Miyake H, Nosaka K, Yoshida M, Ishihara S, Horikawa K, Yonemura Y, Iyama K, Mitsuya H, Asou N Hairy cell leukemia (HCL) is occasionally misdiagnosed as aplastic anemia when only a few leukemic cells are present in the circulation. Here, we describe a patient with HCL who initially presented with pancytopenia and received a diagnosis of aplastic anemia. The patient was treated with immunosuppressive therapy including cyclosporine A and anti-thymocyte globulin (ATG). No blood cell transfusion was required for approximately 3 years after ATG therapy. She was referred to our hospital because of an abdominal mass and requiring periodic blood transfusions. A bone marrow biopsy at this time revealed proliferation of lymphocytes with a fried egg appearance and an increase ...

Late extramedullary relapse after allogeneic transplantation in a case of variant hairy cell leukaemia

Bone Marrow Transplantation — Sun, 11 Oct 2009 23:00:00 +0100

Authors: C Busemann, F Schüler, W Krüger, T Kiefer, M Wuppermann, M Androshchuk & G Dölken (Source: Bone Marrow Transplantation)

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Interferon as an alternative to purine analogues in the treatment of hairy cell leukaemia – response to Benz et al

British Journal of Haematology — Tue, 06 Oct 2009 23:00:00 +0100

(Source: British Journal of Haematology)

Interferon as an alternative to purine analogues in the treatment of hairy cell leukaemia

British Journal of Haematology — Tue, 06 Oct 2009 23:00:00 +0100

(Source: British Journal of Haematology)

Antibody-based therapy of leukaemia

Expert Reviews in Molecular Medicine — Wed, 30 Sep 2009 18:21:02 +0100

Review ArticlesJohn C. Morris, Thomas A. Waldmann, Expert Reviews in Molecular Medicine, Volume 11 , pp e29AbstractOver the past decade, monoclonal antibodies have dramatically impacted the treatment of haematological malignancies, as evidenced by the effect of rituximab on the response rate and survival of patients with follicular and diffuse large B cell non-Hodgkin's lymphoma. Currently, only two monoclonal antibodies are approved for treatment of relapsed acute myeloid leukaemia in older patients and B cell chronic lymphocytic leukaemia, respectively. Although not approved for such treatment, alemtuzumab is also active against T cell prolymphocytic leukaemia, cutaneous T cell lymphoma and S zary syndrome, and adult T cell leukaemia and lymphoma. In addition, rituximab has demonstrated...

VH4-34+ Hairy Cell Leukemia, a New Variant With Poor Prognosis Despite Standard Therapy

OncologySTAT Journal Scans — Tue, 22 Sep 2009 16:16:18 +0100

VH4-34 status relates to poorer response to cladribine and shorter survival among patients with HCLv. Hairy cell leukemia variant (HCLv) presents with high disease burden, lack of typical antigens like... (Source: OncologySTAT Journal Scans)

Hairy cell leukemia presenting as multiple discrete hepatic lesions.

World Journal of Gastroenterology : WJG — Sun, 20 Sep 2009 21:30:18 +0100

Authors: Sahar N, Schiby G, Davidson T, Kneller A, Apter S, Farfel Z The involvement of hairy cell leukemia in the liver is in the form of portal and sinusoidal cellular infiltration. Here we describe the first case of hepatic hairy cell leukemia presenting as multiple discrete lesions, which was treated successfully. We suggest that in the investigation of discrete hepatic lesions in cases of cancer of unknown primary, hairy cell leukemia should be considered. The excellent response of hairy cell leukemia to therapy highlights the need for such a consideration. PMID: 19764101 [PubMed - in process] (Source: World Journal of Gastroenterology : WJG)

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Management of hematological malignancies during pregnancy

American Journal of Hematology — Tue, 15 Sep 2009 23:00:00 +0100

The management of hematological malignancies during pregnancy is a challenging endeavor, which not only requires technical skills and knowledge by the clinicians but also requires sound clinical judgment and compassion, keeping in mind the patient and family preferences and, ultimately, the wellbeing of the neonate. The incidence of hematological malignancies during pregnancy is rare, ranging from 1 in 1,000 to 1 in 10,000 deliveries, impeding the design and execution of large prospective studies. The purpose of this review is to evaluate the limited existing data and make useful suggestions in the management of acute and chronic leukemias, Hodgkin and non-Hodgkin lymphomas, plasma cell myeloma, and other hematological malignancies, such as myelodysplastic syndromes and hairy cell leukemia...

Hairy Cell Leukemia.

Clinical Lymphoma and Myeloma — Mon, 31 Aug 2009 23:00:00 +0100

Authors: Ravandi F Progress in the treatment of patients with hairy cell leukemia (HCL) has led to a significant change in the natural history of the disease. With current regimens, the majority of patients achieve a complete remission, and their survival curves are similar to those for appropriate age-matched individuals without the disease. At the same time, new technologies are allowing better understanding of the molecular mechanisms responsible for the pathogenesis of this and other indolent lymphoid neoplasms. Several studies using modern techniques with different sensitivities have demonstrated the persistence of minimal residual disease (MRD) after therapy with nucleoside analogues in majority of patients. However, it is not clear whether such MRD would invariably lead to leuke...

Characterization of c-Maf transcription factor in normal and neoplastic hematolymphoid tissue and its relevance in plasma cell neoplasia.

American Journal of Clinical Pathology — Thu, 20 Aug 2009 23:52:23 +0100

Authors: Natkunam Y, Tedoldi S, Paterson JC, Zhao S, Rodriguez-Justo M, Beck AH, Siebert R, Mason DY, Marafioti T c-Maf, a leucine zipper-containing transcription factor, is involved in the t(14;16)(q32;q23) translocation found in 5% of myelomas. A causal role for c-Maf in myeloma pathogenesis has been proposed, but data on c-Maf protein expression are lacking. We therefore studied the expression of c-Maf protein by immunohistochemical analysis in myelomas and in a wide variety of hematopoietic tissue. c-Maf protein was detected in a small minority (4.3%) of myelomas, including a t(14;16)(q32;q22-23)/IgH-Maf+ case, suggesting that c-Maf protein is not expressed in the absence of c-Maf rearrangement. In contrast, c-Maf was strongly expressed in hairy cell leukemia (4/4) and in a signifi...

Rare lymphoid malignancies of the breast: a report of two cases illustrating potential diagnostic pitfalls

Journal of Hematopathology — Thu, 20 Aug 2009 08:31:19 +0100

We describe two cases of lymphoid malignancies predominantly involving the breast, both presenting diagnostic dilemmas. The first case, ALK-negative anaplastic large-cell lymphoma involving a seroma associated with a breast implant, is an emerging clinicopathologic entity. Anaplastic large-cell lymphoma has been identified in association with breast implants and seroma formation relatively recently. The second case, hairy cell leukemia involving the breast and ipsilateral axillary sentinel lymph node, is, to our knowledge, the first reported case of hairy cell leukemia involving the breast at the time of diagnosis. While a localized bone lesion was present at time of diagnosis, bone marrow involvement was relatively mild in comparison to that seen in the breast and lymph node. In the...

Removal of B cell epitopes as a practical approach for reducing the immunogenicity of foreign protein-based therapeutics.

Advanced Drug Delivery Reviews — Sun, 09 Aug 2009 23:00:00 +0100

Authors: Nagata S, Pastan I Immunogenicity of non-human proteins with useful therapeutic properties has prevented their development for use in the therapy of disease. However, this class of proteins could be very useful, if their immunogenicity could be markedly reduced so that many treatment cycles could be administered. One approach to reduce the immunogenicity of foreign proteins is to identify B-cell epitopes on the protein and eliminate them by mutagenesis. In this article, theoretical aspects and experimental evidence for the feasibility of B cell epitope removal is reviewed. A special focus is given to our results with deimmunization of recombinant immunotoxins in which Fvs are fused to a 38kDa portion of the bacterial protein, Pseudomonas exotoxin A (PE38). Immunotoxins targeti...

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Acquired Glanzmann's thrombasthenia associated with Hairy cell leukaemia

European Journal of Clinical Investigation — Wed, 08 Jul 2009 23:00:00 +0100

(Source: European Journal of Clinical Investigation)

Novel drugs for chronic lymphoid leukemias: mechanism of action and therapeutic activity.

Current Medicinal Chemistry — Sun, 28 Jun 2009 03:08:03 +0100

Authors: Robak T Chronic lymphoid leukemias include well defined mature B-cell and T-cell neoplasms with diverse natural history and specific morphological, immunophenotypic and molecular characteristics. The most common adult leukemia in the Western world is chronic lymphocytic leukemia (CLL). Rarer indolent lymphoid leukemias include prolymphocytic leukemia, hairy cell leukemia, large granular lymphocyte leukemia and T-cell leukemia/lymphoma. Recently, several new agents have been explored and have shown promise in CLL treatment. Novel therapies are being evaluated both in pre-clinical studies and in early clinical trials. These treatments include new purine nucleoside analogs, antisense oligonucleotides, agents targeting the antiapoptotic Bcl-2 family of proteins, receptors involved...

Risk factors for severe infection in patients with hairy cell leukemia: a long-term study of 73 patients

European Journal of Haematology — Fri, 26 Jun 2009 23:00:00 +0100

Although the survival of patients with hairy cell leukemia (HCL) has been improved by the therapeutic introduction of interferon [alpha] and purine analogs, it is still worsened by complications such as severe infections. In this long-term study, we identified factors influencing patient outcomes in 73 patients with HCL. Median age at diagnosis was 53 yr and the gender ratio (M/F) was 2.3. At the time of HCL diagnosis, 60 patients (82%) were symptomatic and 22 of these had an infection. After a median follow-up of 13 yr, eight patients had died of secondary cancer (n = 2), HCL progression (n = 1) and age-related complications (n = 5). The 10-yr overall survival (OS), progression-free survival and relapse rates were 91 ± 3%, 14 ± 5% and 87 ± 5%, respectively. In multivariate analyses, ag...

Bispecific immunotoxins

Leukemia Research — Fri, 26 Jun 2009 14:24:05 +0100

Chemotherapy resistance is a frequent clinical dilemma in the therapy of many neoplasms. Since most cytotoxic drugs target DNA or cell proliferation, alternate methods of cancer therapy have been sought. A number of laboratories have synthesized and tested clinically immunotoxins. These protein agents consist of peptide cell-selective ligands coupled to catalytic toxins. The toxins damage cells by damaging plasma membranes or inactivating cytosolic protein synthesis. Clinical trials of immunotoxins have shown mixed results. Remissions have been observed with several immunotoxins for hematologic malignancies including denileukin diftitox for T cell lymphomas and BL22 for hairy cell leukemias . However, in many cases immunotoxin therapy has been associated with significant toxicities to norm...

Commentary on the WHO classification of tumors of lymphoid tissues (2008): indolent B cell lymphomas

Journal of Hematopathology — Thu, 25 Jun 2009 06:54:35 +0100

Abstract The 4th edition of the World Health Organization classification of tumors of hematopoietic and lymphoid tissues introduces many new items to the classification scheme of the so-called indolent B cell lymphomas. New proposed entities, such as splenic B cell lymphoma/leukemia, unclassifiable, splenic diffuse red pulp small B cell lymphoma, hairy cell leukemia variant, pediatric follicular lymphoma, and pediatric marginal zone lymphoma have been coined, and some definitions of established diseases, such as chronic lymphocytic leukemia or Waldenström’s macroglobulinemia have been revised. One aspect of major importance is the recent description of small clonal B cell populations, in part with a CLL phenotype, and their relationship to B-CLL. Some new subtypes or vari...

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[Leukemia and Bone Marrow Transplantation] Phase II Trial of Recombinant Immunotoxin RFB4(dsFv)-PE38 (BL22) in Patients With Hairy Cell Leukemia

Journal of Clinical Oncology — Wed, 17 Jun 2009 23:00:00 +0100

Conclusion BL22 activity in HCL is confirmed. Best responses to BL22 after cladribine failure are achieved before the patients develop massive splenomegaly or undergo splenectomy. (Source: Journal of Clinical Oncology)

Specific skin lesions in hairy cell leukemia at presentation: case report and review of literature

Medical Oncology — Wed, 17 Jun 2009 07:16:44 +0100

Abstract Skin involvement in hairy cell leukemia (HCL) at presentation is a relatively rare manifestation of the disease. A 60-year-old male patient in whom cutaneous lesions were the initial manifestation of hairy cell leukemia together with leukocytosis, monocytopenia, massive splenomegaly, and leukemic maculopapulous infiltration of the almost whole skin is described. The present case is the forth mentioned in the literature with specify of leukocytosis in peripheral blood, consisting mostly of hairy cells. The patient was treated with two courses of 2-chlorodeoxiadenosine (2-CdA, Cladribine) and splenectomy and after this cutaneous lesion disappeared and general condition is improved. Content Type Journal ArticleCategory Review ArticleDOI 10.1007/s12032-009-9246-4Aut...

Survival in non-Hodgkin’s lymphoma by histology and family history

Journal of Cancer Research and Clinical Oncology — Wed, 17 Jun 2009 07:05:58 +0100

Conclusions Our results provide quantitative prognosis data for patients with NHL according to specific histologies. Patients with a familial NHL had a similar prognosis compared to patients with sporadic disease. The data suggest familial concordance in either good or poor survival among family members. Content Type Journal ArticleCategory Original PaperDOI 10.1007/s00432-009-0618-0Authors Jianguang Ji, Lund University Center for Primary Health Care Research CRC, hus 28, plan 11, ing 72, UMAS 205 02 Malmö SwedenAsta Försti, Lund University Center for Primary Health Care Research CRC, hus 28, plan 11, ing 72, UMAS 205 02 Malmö SwedenJan Sundquist, Lund University Center for Primary Health Care Research CRC, hus 28, plan 11, ing 72, UMAS 205 02 Malmö SwedenPer Lenn...

[Original articles] Occupational exposure to pesticides and lymphoid neoplasms among men: results of a French case-control study

Occupational and Environmental Medicine — Fri, 17 Apr 2009 04:00:00 +0100

Conclusions: The results, based on case-by-case expert review of occupation-specific questionnaires, support the hypothesis that occupational pesticide exposures may be involved in HL, MM and HCL and do not rule out a role in NHL. The analyses identified specific pesticides that deserve further investigation and the findings were consistent with those of previous studies. (Source: Occupational and Environmental Medicine)

Sweet syndrome as the presenting symptom of hairy cell leukemia.

Dermatol Online J — Tue, 07 Apr 2009 13:34:47 +0100

Authors: Ventura F, Rocha J, Pereira T, Marques H, Pardal F, Brito C PMID: 19336029 [PubMed - in process] (Source: Dermatol Online J)

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[Treatments of Hodgkin and non-Hodgkin lymphomas.]

Orvosi Hetilap — Sun, 05 Apr 2009 04:00:00 +0100

Authors: Sréter L The therapy of malignant lymphoproliferative diseases has changed many times in recent years. Treatment strategy of Hodgkin's disease is now based on risk adaptation, including not only the results of pretreatment diagnostic and prognostic factors but also the repeated PET/CT (restaging) made in the early treatment period. Possible reduction of irradiation therapy may contribute to lower the risk of secondary tumors, which are common late complications of radiochemotherapy. Autologous stem cell transplantation is the therapy of choice in chemosensitive relapsing patients. The complete remission rate today in Hodgkin's disease is around 85%. In the heterogenic group of Non-Hodgkin Lymphomas, progression of indolent lymphomas (CLL, multiple myeloma, hairy cell leuk...

Establishment of a Hairy Cell Leukemia Variant Cell Line, HCLv-07.

Acta Haematologica — Wed, 01 Apr 2009 04:00:00 +0100

Authors: Sasaki M, Aritaka N, Tsukune Y, Kawahara S, Masuda A, Tsutsui M, Kanemitsu N, Sugimoto K PMID: 19332987 [PubMed - as supplied by publisher] (Source: Acta Haematologica)

Achievement of complete remission of refractory hairy cell leukemia by rituximab progressing after allogeneic hematopoietic stem cell transplantation.

International Journal of Hematology — Tue, 31 Mar 2009 04:00:00 +0100

Authors: Kiyasu J, Shiratsuchi M, Ohtsuka R, Sada E, Idutsu K, Nagasawa E, Takayanagi R, Abe Y PMID: 19330554 [PubMed - as supplied by publisher] (Source: International Journal of Hematology)

Long-term follow-up of 233 patients with hairy cell leukaemia, treated initially with pentostatin or cladribine, at a median of 16 years from diagnosis

British Journal of Haematology — Sun, 29 Mar 2009 04:00:00 +0100

Hairy cell leukaemia (HCL) was first described 50 years ago. Median survival was then 4 years. The purine analogues, introduced in the 1980s, transformed this prognosis. We reviewed data retrospectively from 233 patients, treated with pentostatin (n = 188) or cladribine (n = 45), to investigate the current long-term outlook. Median follow-up was 16 years. There were no significant differences in outcome between the two agents. Overall, the complete response (CR) rate was 80% and median relapse-free survival was 16 years. After relapse (n = 79) or non-response (n = 5), 26 patients received pentostatin and 58 cladribine; 69% achieved CR and median relapse-free survival was 11 years. After third-line therapy (n = 23), 50% achieved CR and median relapse-free survival was 6·5 years. However, C...

Immunophenotypic analysis of CD103+ B-lymphoproliferative disorders: hairy cell leukemia and its mimics.

American Journal of Clinical Pathology — Sat, 21 Mar 2009 10:44:55 +0100

Authors: Dong HY, Weisberger J, Liu Z, Tugulea S CD103 is characteristically expressed in hairy cell leukemia (HCL), a B-lymphoproliferative disorder highly responsive to treatment with purine analogs. Other CD103+ diseases are rare and do not respond well to the same therapy, including HCL variant (HCLv) and splenic marginal zone B-cell lymphoma (SMZL) variants. We analyzed 215 cases of CD103+ B-lymphoproliferative disorders to further delineate their immunophenotypic features. Flow cytometric analysis revealed that 78.6% of all cases expressed CD25 and CD103, characteristic of classical HCL. Cases analyzed immunohistochemically were also invariably positive for annexin-A1; a subset coexpressed CD10 (33/169 [19.5%]) or BCL1 (26/65 [36.9%]). In contrast, 21.4% of cases lacked CD25, a s...

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Hairy Cell Leukemia-Related Disorders Consistently Show Low CD27 Expression.

Pathology Oncology Research — Fri, 20 Mar 2009 04:00:00 +0100

Authors: Hashimoto Y, Tsukamoto N, Nakahashi H, Yokohama A, Saitoh T, Handa H, Matsushima T, Murakami H, Nojima Y, Karasawa M In Japan, typical hairy cell leukemia (HCL) is rare, and HCL-Japanese variant (HCL-JV) is more common. Hairy B-cell lymphoproliferative disorder (HBLD) is another unusual disorder of polyclonal B-lymphocytosis of hairy cell appearance. In the present study, we analyzed the clinical features of 3 patients with HCL, 3 with HCL-JV, and 3 with HBLD. All HBLD patients had the DRB1*04 allele. As compared with other B-cell lymphoproliferative disorders, CD27 expression on B cells was significantly lower in all patients, ranging from 0.3% to 23.4%. Our results suggest that low CD27 expression may be a distinct feature of these HCL-related disorders. PMID: 19301150 [...

Has Success Spoiled Hairy Cell Leukemia Research? Key Questions Go Unanswered, Despite Big Gains

JNCI — Tue, 17 Mar 2009 04:00:00 +0100

(Source: JNCI)

Elevated risk of chronic lymphocytic leukemia and other indolent non-Hodgkin's lymphomas among relatives of patients with chronic lymphocytic leukemia.

Haematologica — Fri, 13 Mar 2009 04:00:00 +0100

Conclusions These familial aggregations are striking and provide novel clues to research designed to uncover early pathogenetic mechanisms in chronic lymphocytic leukemia including studies to identify germ line susceptibility genes. However, clinicians should counsel their chronic lymphocytic leukemia patients emphasizing that because the baseline population risks are low, the absolute risk for a first-degree relative to develop chronic lymphocytic leukemia or another indolent lymphoma is low. At this time, an increased medical surveillance of first-degree relatives of chronic lymphocytic leukemia patients has no role outside research studies. PMID: 19286886 [PubMed - as supplied by publisher] (Source: Haematologica)

CD200/OX2, a cell surface molecule with immuno-regulatory function, is consistently expressed on hairy cell leukaemia neoplastic cells

British Journal of Haematology — Thu, 12 Mar 2009 04:00:00 +0100

(Source: British Journal of Haematology)

Visual disturbance as initial presentation of hairy cell leukemia.

European Journal of Ophthalmology — Sun, 01 Mar 2009 05:00:00 +0100

CONCLUSIONS. The authors present a rare case in which bilateral visual complaints led to the diagnosis of HCL. Thus, though uncommon, HCL should be considered in the differential diagnosis of otherwise unexplained retinal hemorrhages. PMID: 19253258 [PubMed - in process] (Source: European Journal of Ophthalmology)

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CD26 expression in mature B-cell neoplasia: its possible role as a new prognostic marker in B-CLL

Hematological Oncology — Thu, 26 Feb 2009 05:00:00 +0100

CD26 (dipeptidyl peptidase IV, DPP IV) is widely expressed by T and natural killer (NK) cells, epithelial and endothelial cells of different tissues, and it is strongly upregulated in activated B-cells; moreover it plays a regulatory role in the neoplastic transformation and progression of various types of tumours. CD26 expression was evaluated by means of flow cytometry in various peripheral B-cell lymphoid tumours: 12 follicular and 12 mantle cell lymphomas, 20 multiple myelomas (MMs), 12 hairy cell leukaemias (HCLs), 112 chronic lymphocytic leukaemias (CLLs), 20 CD5negative B-cell chronic lymphoproliferative diseases (CD5neg B-CLPDs) and 12 diffuse large cell lymphomas (DLCLs). CD26 expression was absent or barely detectable in follicular and mantle cell lymphomas, high in MMs and HCLs,...

[Correspondence] Teenager With Hairy Cell Leukemia: 30-Year Follow-Up

Journal of Clinical Oncology — Mon, 29 Dec 2008 05:00:00 +0100

(Source: Journal of Clinical Oncology)

Hairy-Cell Leukemia: 50 Years Of Research

Lymphoma / Leukemia News From Medical News Today — Mon, 08 Dec 2008 08:00:00 +0100

In 1958, Ohio State University cancer researcher Dr. Bertha Bouroncle first identified a deadly disease now known as hairy-cell leukemia, a once fatal disease that can now be effectively treated. Now, 50 years later researchers from across the globe are gathering for a symposium titled "50 years of Enormous Progress in Hairy Cell Leukemia: A Celebration of Clinical Research with Remaining Unanswered Questions. (Source: Lymphoma / Leukemia News From Medical News Today)

50 Years Of Hairy-Cell Leukemia Research To Be Observed

Huliq Health News — Sat, 06 Dec 2008 07:12:31 +0100

50 years of hairy-cell leukemia research to be observed

EurekAlert! - Cancer — Fri, 05 Dec 2008 05:00:00 +0100

(Ohio State University Medical Center) In 1958, Ohio State University cancer researcher Dr. Bertha Bouroncle first identified a deadly disease now known as hairy-cell leukemia, a once fatal disease that can now be effectively treated. Now, 50 years later researchers from across the globe are gathering for a symposium titled "50 years of Enormous Progress in Hairy Cell Leukemia: A Celebration of Clinical Research with Remaining Unanswered Questions." (Source: EurekAlert! - Cancer)

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Xanthones from the Seeds of Allanblackia monticola and Their Apoptotic and Antiproliferative Activities

Planta Medica — Thu, 04 Dec 2008 02:39:50 +0100

Planta MedDOI: 10.1055/s-0028-1088375AbstractPhytochemical investigations of the seeds of have led to the isolation and characterization of one new xanthone derivative, named allanxanthone E (), together with seven known compounds, including five xanthones, 1,7-dihydroxy-3-methoxy-2-(3-methylbut-2-enyl)xanthone (), -mangostin () garciniafuran () allanxanthone C (), and 1,6-dihydroxy-2,4-diprenylxanthone (), and two pentacyclic triterpenes, friedelin and lupeol. The structures of these compounds were established on the basis of one- and two-dimensional NMR homo- and heteronuclear correlation evidence. Some of these compounds were evaluated for their apoptotic and antiproliferative activities against human leukemic B lymphocytes, such as the hairy cell leukemia-derived ESKOL cell line and ce...

Non-traumatic splenic rupture: Report of seven cases and review of the literature.

World Journal of Gastroenterology : WJG — Fri, 21 Nov 2008 05:00:00 +0100

CONCLUSION: NSR is a rare entity that needs a high index of suspicion for diagnosis. Using ultrasonography or computer tomography, and peritoneal aspiration of fresh blood may assist in the diagnosis of NSR. Increased awareness of NSR can enhance early diagnosis and effective treatment. PMID: 19034976 [PubMed - in process] (Source: World Journal of Gastroenterology : WJG)

Hairy Cell Leukemia with Unusual Loss of CD103 in a Subset of the Neoplastic Population: Immunophenotypic and Cell Cycle Analysis by Flow Cytometry.

International Journal of Clinical and Experimental Pathology — Sun, 02 Nov 2008 19:20:04 +0100

We report an unusual case of hairy cell leukemia (HCL) in a 55-year-old male who presented with fatigue, increased bruising, leukocytosis, anemia, thrombocytopenia and moderate splenomegaly without lymphadenopathy. Microscopically, a monomorphic population of small to medium-sized lymphoid cells with bean-shaped nuclei, ground glass chromatin and fine cytoplasmic projections was identified in the peripheral blood and bone marrow. Flow cytometric immunophenotyping demonstrated a monoclonal population of mature B cells with coexpression of CD25, CD11c and CD103. The clonal B-cells all exhibited homogenous expression of CD20 and uniform light scatter characteristics. However, CD103 expression was present in only half of the clonal B-cells. Flow cytometric cell cycle analysis using DRAQ5 DNA d...

Mycobacterium kansasii in a patient with hairy cell leukaemia

British Journal of Haematology — Thu, 16 Oct 2008 04:00:00 +0100

(Source: British Journal of Haematology)

Paraneoplastic scleroderma secondary to hairy cell leukaemia successfully treated with cladribine

Rheumatology — Thu, 16 Oct 2008 04:00:00 +0100

(Source: Rheumatology)

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Extranodal hairy cell leukemia presenting in the lumbar spine.

Journal of Neurosurgery.Spine — Wed, 01 Oct 2008 04:00:00 +0100

Authors: Rosen DS, Smith S, Gurbuxani S, Yamini B The authors report on a 54-year-old man who presented with a lumbar vertebral body lesion and an adjacent epidural lesion that was found to be hairy cell leukemia (HCL). The patient presented with gradual onset of back pain and intermittent lower-extremity radicular symptoms. He did not have splenomegaly or peripheral blood count abnormalities. Admission MR imaging revealed an L-5 vertebral body lesion and a lumbar epidural lesion extending from L-3 to S-2. An [18F]fluorodeoxyglucose-PET study showed numerous sites of osseous involvement. The patient underwent minimally invasive surgical biopsy sampling of the epidural lesion. Histopathological examination revealed extranodal HCL. After treatment with a 5-day course of cladribine, the p...

The small GTPase RhoH is an atypical regulator of haematopoietic cells

Cell Communication and Signaling — Mon, 29 Sep 2008 04:00:00 +0100

Rho GTPases are a distinct subfamily of the superfamily of Ras GTPases. The best-characterised members are RhoA, Rac and Cdc42 that regulate many diverse actions such as actin cytoskeleton reorganisation, adhesion, motility as well as cell proliferation, differentiation and gene transcription. Among the 20 members of that family, only Rac2 and RhoH show an expression restricted to the haematopoietic lineage. RhoH was first discovered in 1995 as a fusion transcript with the transcriptional repressor LAZ3/BCL6. It was therefore initially named translation three four (TTF) but later on renamed RhoH due to its close relationship to the Ras/Rho family of GTPases. Since then, RhoH has been implicated in human cancer as the gene is subject to somatic hypermutation and by the detection of RHOH as...

Cigarette smoking, alcohol drinking, and risk of lymphoid neoplasms: results of a French case–control study

Cancer Causes and Control — Wed, 10 Sep 2008 07:05:32 +0100

Conclusion Results are consistent with those of several previous studies and suggest a direct or indirect protective effect of smoking with respect to HCL although based on small numbers. The negative relationship between alcohol consumption and Hodgkin’s and NHL, also previously reported, needs further investigations. Content Type Journal ArticleCategory Original PaperDOI 10.1007/s10552-008-9182-1Authors A. Monnereau, Inserm U754 94800 Villejuif Cedex FranceL. Orsi, Inserm U754 94800 Villejuif Cedex FranceX. Troussard, Côte de Nacre Hospital Caen FranceC. Berthou, Morvan Hospital Brest FranceP. Fenaux, Avicenne Hospital Bobigny FranceP. Soubeyran, Bergonié Institute Comprehensive Cancer Center 33076 Bordeaux Cedex FranceG. Marit, Haut-Lévêque Hospital, CHU Bord...

Soluble CD22 as a tumor marker for hairy cell leukemia

Blood — Mon, 08 Sep 2008 04:00:00 +0100

CD22 is an important immunotherapeutic target on B-cell malignancies, particularly hairy cell leukemia (HCL), but its soluble extracellular domain, sCD22, has not yet been reported in the blood. By immunoaffinity and enzyme-linked immunosorbent assay techniques using anti-CD22 monoclonal antibodies, we identified the 100-kDa extracellular domain of CD22 and an 80-kDa processed form in serum of patients with HCL. The median sCD22 level measured by enzyme-linked immunosorbent assay was 18 ng/mL for 93 patients with HCL. sCD22 levels varied from 2.1 to 163 ng/mL and were higher (P < .001) than 23 normal donors (median, 0.6 ng/mL). More than 95% of normal donors had sCD22 levels less than 1.9 ng/mL. sCD22 levels were proportional to concentrations of circulating HCL cells (P = .002), and HC...