MedWorm: Hairy Cell Leukemia

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)

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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 ...

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)

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...

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CC Grand Rounds: (1) Human Subjects Research and the Internet Era (2) Updates on Therapies for Hairy Cell Leukemia

Videocast - All Events — Thu, 07 Jan 2010 13:32:00 +0100

Presented by: NIH Clinical CenterCategory: Clinical Center Grand RoundsAired date: 01/06/2010 (Source: Videocast - All Events)

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...

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)

CC Grand Rounds: (1) Human Subjects Research and the Internet Era (2) Updates on Therapies for Hairy Cell Leukemia

Videocast - All Events — Wed, 23 Dec 2009 16:27:00 +0100

Robert Martensen, MD, PhD, Director, Office of History and Museum, NIH Robert Kreitman, MD, Chief, Clinical Immunotherapy Section, Laboratory of Molecular Biology, NCI For more information, visit http://www.cc.nih.gov/about/news/grcurrent.htmlAir date: 1/6/2010 12:00:00 PM (Source: Videocast - All Events)

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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...

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...

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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...

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)

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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)

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...

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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)

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...

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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...

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...

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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...

[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...

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[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)

[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)

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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...

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)

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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)

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)

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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)

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...

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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)

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...

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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...

Effective treatment of a refractory hairy cell leukemia variant with splenic pre-irradiation and alemtuzumab.

Acta Haematologica — Fri, 05 Sep 2008 10:12:42 +0100

Authors: Sasaki M, Sugimoto K, Mori T, Karasawa K, Oshimi K A 72-year-old Japanese man presented with 43.1 x 10(9)/l hairy cells and apparent splenomegaly. The leukemia cells had unevenly distributed microvilli and round nuclei with dense chromatin and one or two clear nucleoli, lacked CD25 expression and were negative for tartrate-resistant acid phosphatase. The case was diagnosed as hairy cell leukemia variant (HCLv) and proved refractory to various chemotherapies, including cladribine, pentostatin, interferon-alpha, CHOP and rituximab. Because of the CD52 expression, we treated the patient with alemtuzumab. Pretreatment with 22.5 Gy to the spleen reduced the spleen size from 12 to 4 cm below the left costal margin, and the number of circulating leukemic cells decreased from 229.0 to...

Rituximab as treatment for minimal residual disease in hairy cell leukaemia: extended follow-up

British Journal of Haematology — Fri, 15 Aug 2008 04:00:00 +0100

(Source: British Journal of Haematology)

Defective interferon-gamma production in patients with hairy cell leukaemia.

The Netherlands Journal of Medicine — Fri, 01 Aug 2008 04:00:00 +0100

Conclusion: The specific defect in IFNγ; production may play a role in the susceptibility of patients with hairy cell leukaemia towards intracellular pathogens. PMID: 18809981 [PubMed - as supplied by publisher] (Source: The Netherlands Journal of Medicine)

Rituximab in patients with hairy cell leukemia relapsing after treatment with 2-chlorodeoxyadenosine (SAKK 31/98).

Haematologica — Fri, 04 Jul 2008 04:00:00 +0100

Authors: Zenhäusern R, Simcock M, Gratwohl A, Hess U, Bargetzi M, Andreas T, PMID: 18603561 [PubMed - as supplied by publisher] (Source: Haematologica)

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Predictive value of blood and bone marrow flow cytometry in B-cell lymphoma classification: comparative analysis of flow cytometry and tissue biopsy in 252 patients.

Mayo Clinic Proceedings — Tue, 01 Jul 2008 04:00:00 +0100

CONCLUSION: Stringent interpretation of PB and bone marrow FCIP results enables identification of certain B-cell lymphoma types. However, the observed exceptions to accepted immunophenotypic paradigms highlight the occasional phenotypic overlap among diseases and emphasize that a systematic approach to FCIP interpretations is key to providing clinically useful diagnostic information. PMID: 18613994 [PubMed - in process] (Source: Mayo Clinic Proceedings)

A Novel CD11c Monoclonal Antibody Effective in Formalin-Fixed Tissue for the Diagnosis of Hairy Cell Leukemia

Pathobiology — Thu, 26 Jun 2008 15:17:36 +0100

Pathobiology 2008;75:252-256 (DOI:10.1159/000132386) (Source: Pathobiology)

Underexpression of RhoH in Hairy Cell Leukemia

Cancer Research — Mon, 16 Jun 2008 04:00:00 +0100

The cause of hairy cell leukemia (HCL) is unknown. Current treatments seem effective only for a limited period of time. In addition, a significant proportion of patients remain refractive to all treatment options. These considerations indicate the need to develop alternative therapeutic strategies for HCL. Here, we report that HCL is characterized by underexpression of RhoH. In vitro reconstitution of RhoH expression inhibits the aberrant adhesion and transendothelial migration that drives disease pathogenesis. In an in vivo model of HCL, RhoH reconstitution limits malignant progression and protects against mortality. These findings provide the proof of principle that RhoH reconstitution represents a potential new approach to the treatment of HCL. [Cancer Res 2008;68(12):4531–40] (So...

[Commentary] Hairy Cell Leukemia: Treatment Successes in the Past 25 Years

Journal of Clinical Oncology — Wed, 28 May 2008 04:00:00 +0100

(Source: Journal of Clinical Oncology)

Chimeric monoclonal anti-CD20 antibody (rituximab)—an effective treatment for a patient with relapsing hairy cell leukaemia

Cancer Immunology, Immunotherapy — Sat, 10 May 2008 09:16:19 +0100

Abstract A case story is presented, describing a 46 y old man, with a relapsing hairy cell leukaemia. After treatment with monoclonal anti CD-20 antibodies (rituximab) 375 mg/week, four times, a complete remission was obtained which has lasted >9 months. The rituximab treatment produced a better remission than earlier treatments with alpha-interferon and chlorodeoxyadenosine. In addition, in contrast to other treatments, no initial worsening of the pancytopenia was observed. Content Type Journal ArticleCategory Case ReportDOI 10.1007/BF02906136Authors H Hagberg, Akademiska sjukhuset Department of Oncology 75185 Uppsala Sweden Journal Cancer Immunology, ImmunotherapyOnline ISSN 1432-0851Print ISSN 0340-7004 Journal Volume Volume 16 Journal Issue Volume 1...

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Hairy cell leukaemia: a heterogeneous disease?

British Journal of Haematology — Fri, 09 May 2008 17:10:47 +0100

British Journal of Haematology, Volume 0, Issue 0, Page ???, OnlineEarly Articles. SummaryThe US National Cancer Institute’s Surveillance, Epidemiology and End Results program was used to develop aetiological clues for hairy cell leukaemia (HCL). Descriptive techniques (age-adjusted incidence trends, age-specific incidence rates (IR), ... (Source: British Journal of Haematology)

Association between the proliferative rate of neoplastic B cells, their maturation stage, and underlying cytogenetic abnormalities in B-cell chronic lymphoproliferative disorders: analysis of a series of 432 patients

Blood — Thu, 08 May 2008 04:00:00 +0100

Limited knowledge exists about the impact of specific genetic abnormalities on the proliferation of neoplastic B cells from chronic lymphoproliferative disorders (B-CLPDs). Here we analyze the impact of cytogenetic abnormalities on the proliferation of neoplastic B cells in 432 B-CLPD patients, grouped according to diagnosis and site of sampling, versus their normal counterparts. Overall, proliferation of neoplastic B cells highly varied among the different B-CLPD subtypes, the greatest numbers of proliferating cells being identified in diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL). Compared with normal B cells, neoplastic B-CLPD cells showed significantly increased S + G2/M-phase values in mantle cell lymphoma (MCL), B-chronic lymphocytic leukemia (B-CLL), BL, and some D...

High density genome-wide DNA profiling reveals a remarkably stable profile in hairy cell leukaemia

British Journal of Haematology — Mon, 07 Apr 2008 21:46:43 +0100

British Journal of Haematology, Volume 0, Issue 0, Page ???, OnlineEarly Articles. SummaryHairy cell leukaemia (HCL) is a rare B-cell neoplasm for which the molecular mechanisms are largely unknown. High-density genome-wide DNA profiling was performed with Affymetrix 250K arrays to analyse copy number (CN) changes and loss of ... (Source: British Journal of Haematology)

Selective influences in the expressed immunoglobulin heavy and light chain gene repertoire in hairy cell leukemia.

Haematologica — Wed, 02 Apr 2008 04:00:00 +0100

Conclusions Overall, biased IGV usage, preference for Iglambda with universal IGLJ3 usage and a high incidence of LCDR3 homologous motifs suggest selective influences on the B-cell receptor of hairy cell leukemia. Ongoing mutations and isotype switching suggest that influences occur on the tumor B-cell receptor at ectopic sites. PMID: 18387977 [PubMed - as supplied by publisher] (Source: Haematologica)

Erythema Nodosum in Association With Newly Diagnosed Hairy Cell Leukemia and Group C Streptococcus Infection.

The American Journal of Dermatopathology — Sat, 22 Mar 2008 09:27:28 +0100

Page: 160DOI: 10.1097/DAD.0b013e3181618a8aAuthors: Patel, Rishi R MD *; Kirkland, Eugene B BS *; Nguyen, Dennis H MD *; Cooper, Brenda W MD +; Baron, Elma D MD *; Gilliam, Anita C MD, PhD ++ (Source: The American Journal of Dermatopathology)

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Pituitary gland involvement by a gamma delta hepatosplenic lymphoma, a mimicker of pituitary adenoma: report of a rare case

Journal of Neuro-Oncology — Tue, 04 Mar 2008 08:25:39 +0100

Abstract The authors report an unusual case of hepatosplenic T-cell lymphoma in a 41-year-old male patient. He presented initially with low grade fever, hepato-splenomegaly and pancytopenia. Splenectomy was done which showed infiltration of red pulp by monomorphic lymphocytes. Liver was also infiltrated with similar cells. A provisional diagnosis of hairy cell leukemia was made. Subsequently, after 6 months he was found to have a sellar mass, which on microscopy revealed lymphoma cells. These cells were positive for leukocyte common antigen and T-cell markers. Finally, based on overall clinical, histomorphological and immunophenotypic features, a diagnosis of hepatosplenic T-cell lymphoma, possibly gamma delta type, involving pituitary gland was established. On follow...

Reaction Patterns of TRAP and DBA.44 in Hairy Cell Leukemia, Hairy Cell Variant, and Nodal and Extranodal Marginal Zone B-cell Lymphomas.

Applied Immunohistochemistry & Molecular Morphology — Thu, 28 Feb 2008 08:47:44 +0100

Page: 135DOI: 10.1097/PAI.0b013e3180471fd4Authors: Dunphy, Cherie H. MD (Source: Applied Immunohistochemistry & Molecular Morphology)

PRAME expression in hairy cell leukemia.

Leukemia Research — Thu, 21 Feb 2008 05:00:00 +0100

Authors: Arons E, Suntum T, Margulies I, Yuan C, Stetler-Stevenson M, Kreitman RJ PRAME has been proposed as a useful marker for solid tumors and acute B-cell malignancies. Several studies demonstrate expression in CLL. To further examine its B-cell tumor distribution, we studied PRAME in both CLL and hairy cell leukemia (HCL). While by conventional PCR only 8% of 37 HCL and 27% of 22 CLL patients were positive, nearly all patients and normal donors expressed PRAME by real-time quantitative (TaqMan) PCR. We conclude that HCL and CLL differ in PRAME overexpression, and that basal normal expression of PRAME may limit its usefulness for following patients with minimal residual CLL or HCL. PMID: 18295331 [PubMed - as supplied by publisher] (Source: Leukemia Research)

Valganciclovir prevents cytomegalovirus reactivation in patients receiving alemtuzumab-based therapy

Blood — Fri, 08 Feb 2008 05:00:00 +0100

In conclusion, this agent was highly effective for prophylaxis of CMV reactivation in patients receiving alemtuzumab. This trial was registered at www.ClinicalTrials.gov as #NCT00562770. (Source: Blood)

Splenic red pulp lymphoma with numerous basophilic villous lymphocytes: a distinct clinicopathologic and molecular entity?

Blood — Fri, 08 Feb 2008 05:00:00 +0100

The presence of circulating villous lymphocytes (VLs) in lymphoma patients usually points to splenic marginal zone B-cell lymphoma (SMZL), even if the VLs can be found occasionally in other small B-cell lymphomas. However, those cells are variably described, and detailed cytologic characterization is often lacking. We identified lymphoma cases with numerous basophilic VLs among the large group of splenic lymphoma with VLs, and for further delineation, 37 cases with this particular cytology were analyzed. Patients, predominantly older men, presented with moderate lymphocytosis and splenomegaly without pancytopenia. The monoclonal B cells expressed IgM + D, IgM + G, IgM or IgG, as well as CD76 and CD11c, frequently CD103, and rarely CD123. Spleen sections were peculiar, with atrophic white p...

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Hairy cell leukemia

MayoClinic.com Full Feed — Sat, 02 Feb 2008 06:00:00 +0100

— Comprehensive overview covers treatment, diagnosis, symptoms of this rare blood cancer.Sponsored by:Chemotherapy.com - http://www.chemotherapy.com (Source: MayoClinic.com Full Feed)

Hairy cell leukemia presenting as a discrete liver mass: Diagnosis by fine needle aspiration biopsy

Diagnostic Cytopathology — Mon, 07 Jan 2008 05:00:00 +0100

We report a case of hairy cell leukemia associated with a liver mass 20 years after the initial presentation. To our knowledge, this is the first case of hairy cell leukemia presenting as a solid liver mass diagnosed by a percutaneous ultrasound guided fine needle aspiration biopsy to be reported in the English literature. Diagn. Cytopathol. 2008;36:128-132. © 2008 Wiley-Liss, Inc. (Source: Diagnostic Cytopathology)

Splenic lymphoma with villous lymphocytes.

Indian Journal of Pathology and Microbiology — Tue, 01 Jan 2008 05:00:00 +0100

Authors: Gupta R, Naseem S, Sukumaran S, Kashyap R, Kaur S, Paul L Splenic lymphoma with villous lymphocytes (SLVL) is a rare disorder that comprises less than 1% of lymphoid neoplasms. It is the leukemic counterpart of splenic marginal zone lymphoma (SMZL) and is characterized by splenomegaly, often with no lymphadenopathy, moderate lymphocytosis and villous lymphocytes on peripheral blood smear. Here, we report a case of SLVL in a 56-year-old male with very high leukocyte counts, massive splenomegaly and relatively few leukemic cells with subtle villous projections on the surface. This disorder is often confused with other chronic lymphoproliferative disorders, especially chronic lymphocytic leukemia (CLL) and hairy cell leukemia and should be differentiated from them. We are reporti...

FCRL1 on chronic lymphocytic leukemia, hairy cell leukemia, and B-cell non-Hodgkin lymphoma as a target of immunotoxins

Blood — Fri, 21 Dec 2007 05:00:00 +0100

FCRL1 (Fc receptor–like 1) is a cell-surface membrane protein belonging to FCRL family and is preferentially expressed on B cells. To evaluate FcRL1 as an immunotherapy target for B-cell malignancies, we prepared anti-FCRL1 mAbs without cross-reactivity to other FCRL family proteins and analyzed FCRL1 protein expression on malignant cells from patients and on B-cell lines. Frequent FCRL1 expression was observed by flow cytometry on 12 B-cell non-Hodgkin lymphoma (B-NHL) cell lines and many patient samples: 12 of 14 chronic lymphocytic leukemia (CLL), 7 of 7 follicular lymphoma (FL), 13 of 17 hairy cell leukemia (HCL), and 2 of 3 mantle cell lymphoma (MCL). Two recombinant immunotoxins, E3(Fv)-PE38 and E9(Fv)-PE38, were constructed. Both immunotoxins bound to FCRL1-positive cells with...

Partial remission of a newly diagnosed diffuse large B-cell Non-Hodgkin's lymphoma in a hemodialysis patient after administration of immuno-chemotherapy with rituximab-CHOP.

International Journal of Laboratory Hematology — Sat, 01 Dec 2007 05:00:00 +0100

Authors: Feldmann G, Nattermann J, Gerhardt T, Nähle CP, Spengler U, Woitas R To date little data exist about treatment of hematologic malignancies in patients with end-stage renal disease (ESRD). While administration of immunochemotherapy comprising the CD20-antibody rituximab is a well-established treatment strategy in patients with normal renal function, little information on safety and efficacy is available in the setting of ESRD. Here we describe for the first time a hemodialysis patient suffering from diffuse large B-cell Non-Hodgkin's lymphoma (DLBCL) who was treated with polychemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone) in combination with rituximab (R-CHOP). We observed no major adverse events and treatment resulted in a partial remission of the...

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Molecular characterization of complete and incomplete immunoglobulin heavy chain gene rearrangements in hairy cell leukemia.

Clinical Lymphoma and Myeloma — Thu, 01 Nov 2007 04:00:00 +0100

Conclusion: These results support that HCL is a heterogeneous entity including subgroups with different molecular characteristics, which reinforces the need for additional studies with a larger number of patients to clarify the real role of gene rearrangements in HCL. PMID: 18186965 [PubMed - in process] (Source: Clinical Lymphoma and Myeloma)

Hairy Cell Leukemia

Current Treatment Options in Oncology — Wed, 10 Oct 2007 16:41:57 +0100

Opinion Statement Hairy cell leukemia (HCL) is an indolent B-cell lymphoproliferative disease, characterized by splenomegaly and pancitopenia related to this. The lymphocytes present characteristic citoplasmatic projections and are positive for tartrate-resistant acid phosphatase (TRAP). Immunophenotyping is necessary to identify the co expression of CD103, CD25, CD11c associated with a typical B-cell clonally pattern and to make a differential diagnosis from other indolent malignancies. Despite the indolent clinical course, treatment is required to resolve symptoms related to splenomegaly and to reduce the incidence of severe infections that are the major complications and a common cause of death. In the past the treatment was only able to resolve the symptoms. In the rev...

A single unit lymphoma experience - Outcome in a Cape Town academic centre.

Transfusion and Apheresis Science — Tue, 09 Oct 2007 04:00:00 +0100

Authors: Wood L, Robinson R, Gavine L, Juritz J, Jacobs P To document outcome in Hodgkin and other lymphomas from a privately based academic centre the clinical records from 253 consecutive referrals were analysed. Diagnosis was according to World Health Organization criteria, prognosis assigned by the international index and therapy risk-stratified with results subject to appropriate statistical methodology. None of these patients underwent transplantation. For the cohort the median age was 55 years (range 11-94) and 63% were male. Constitutional symptoms were present in 22%; a quarter had previous chemotherapy and a third some form of irradiation prior to referral. Fifty-seven percent were stage I or II and 21% had nodal disease above and below the diaphragm whilst in the remainder c...

A case of Mollitias and Fragilitas Ossium – unusual presentation of hairy cell leukaemia followed by the diagnosis of nonsecretory myeloma

Clinical and Laboratory Haematology — Mon, 08 Oct 2007 09:19:42 +0100

This report describes a patient that presented with leucocytosis and splenomegaly. The abnormal leucocytes showed typical morphology and expressed CD103, CD11c, CD19... (Source: Clinical and Laboratory Haematology)

[PostScript] Polyarteritis nodosa in hairy cell leukaemia: an autopsy report

Journal of Clinical Pathology — Fri, 28 Sep 2007 04:00:00 +0100

(Source: Journal of Clinical Pathology)

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The role of rituximab in combination with pentostatin or cladribine for the treatment of recurrent/refractory hairy cell leukemia

Cancer — Thu, 20 Sep 2007 04:00:00 +0100

The purine analogs pentostatin and cladribine have revolutionized the treatment of hairy cell leukemia (HCL) with overall responses in greater than 85% of patients and a median progression-free survival of up to 15 years. They continue to be effective at second- and even third-line therapy; however, alternative treatments are needed for patients who are or have become refractory to these agents or whose remissions are shorter with each course of therapy.The authors conducted a retrospective review of 8 patients who received pentostatin or cladribine combined concurrently (n = 6 patients) or sequentially (n = 2 patients) with rituximab at second-line therapy (n = 3 patients) and at subsequent lines of therapy (n = 5 patients). Results from a previously reported database of 219 patients with...

Antitumor Activity of Fludarabine against Human Multiple Myeloma in vitro and in vivo

European Journal of Haematology — Sat, 15 Sep 2007 17:39:12 +0100

European Journal of Haematology Volume 0, Issue ja. Abstract Fludarabine, a nucleoside analogue, plays a major role in the treatment of B-cell lymphocytic leukemia, hairy cell leukemia, and indolent lymphomas. There is a controversy about antitumor activity of fludarabine in multiple myeloma. The aim of th... (Source: European Journal of Haematology)

[Successful alemtuzumab treatment of a patient with atypical hairy cell leukaemia variant.]

Orvosi Hetilap — Sat, 01 Sep 2007 04:00:00 +0100

Authors: Telek B, Batár P, Udvardy M Although hairy cell leukaemia and hairy cell leukaemia variant are characterized by much alike clinical features, these two diseases are disparate in nature and treatment. While hairy cell leukaemia responds quite well to 2-chlorodeoxyadenosine (cladribine) treatment, hairy cell leukaemia variant has much worse response rate and has no effective treatment option yet. With other treatment modalities, including monoclonal antibody treatment, we have less experience. Alemtuzumab (Campath-1H, MabCampath) treatment has been reported in a case with hairy cell leukaemia in relaps while there is no data with alemtuzumab therapy in the treatment of hairy cell leukaemia variant. The authors present their case of a 58 year-old male who has been diagnosed ...

Hairy cell leukemia

Medical Oncology — Mon, 13 Aug 2007 19:32:24 +0100

Abstract Hairy cell leukemia (HCL) is a chronic B-cell lymphoproliferative disorder characterized by pancytopenia and variable infiltration of the reticuloendothelial system with “hairy” lymphocytes. HCL is more common in men than women and has a median age of diagnosis of 52 yr. Typically, patients with HCL respond well to purine analog-based therapy. The purpose of this review will be to establish the current status of HCL with respect to its pathophysiology, diagnosis, management, and future directions. Content TypeJournal Article JournalMedical OncologyOnline ISSN 1559-131XPrint ISSN 1357-0560 Journal VolumeVolume 24 Journal IssueVolume 24, Number 1 / January, 2007 (Source: Medical Oncology)

T-bet Transcription Factor Detection Facilitates the Diagnosis of Minimal Hairy Cell Leukemia Infiltrates in Bone Marrow Trephines.

The American Journal of Surgical Pathology — Fri, 27 Jul 2007 00:49:18 +0100

Page: 1181DOI: 10.1097/PAS.0b013e318031045bAuthors: Johrens, Korinna MD; Stein, Harald MD; Anagnostopoulos, Ioannis MD (Source: The American Journal of Surgical Pathology)

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Treatment options in skeletal localizations of hairy cell leukemia: A systematic review on the role of radiation therapy

American Journal of Hematology — Tue, 24 Jul 2007 04:00:00 +0100

Skeletal localizations are a rare complication in hairy cell leukaemia patients, with an extimated incidence of 3%. These lesions, mainly osteolytic, can occur at various sites and are almost always symptomatic. Localized radiation therapy (RT) has been extensively used as effective palliative treatment in such cases, with different total doses and fractionation schedules. In this article, a systematic review of all reported cases with osseous complications is presented, to underline the role of RT and to define the most appropriate approach in this subset of patients. Am. J. Hematol., 2007. © 2007 Wiley-Liss, Inc. (Source: American Journal of Hematology)

Weekly cladribine treatment is comparable to daily treatment in active hairy cell leukaemia

Inpharma — Mon, 18 Jun 2007 14:34:28 +0100

(Source: Inpharma)

Weekly cladribine treatment is comparable to daily treatment in active hairy cell leukaemia.

Inpharma Weekly — Sun, 17 Jun 2007 07:26:11 +0100

Page: 16 (Source: Inpharma Weekly)

Acute vasculitis as a first manifestation of hairy cell leukemia.

European Journal of Internal Medicine — Tue, 01 May 2007 04:00:00 +0100

We report an unusual case of HCL in a 53-year-old woman with leukopenia and sudden onset of fever of unknown origin, arthritis, and generalized maculopapular exanthem. Skin biopsy revealed perivascular and/or vessel wall lymphocytic infiltration in the dermis. On the basis of bone marrow biopsy and flow cytometry, the diagnosis of HCL was established. A detailed, retrospective re-evaluation of the skin biopsy helped to identify hairy cells among the cells of the perivascular infiltrations. Small-vessel vasculitis, as an atypical presentation, was found to predate a diagnosis of HCL. Recognition that vasculitis can reflect or antedate lymphoid malignancy may permit early diagnosis and aggressive treatment. A rapid response was obtained with a single course of cladribine. PMID: 17449398 ...

Cladribine in a weekly versus daily schedule for untreated active hairy cell leukemia: final report from the Polish Adult Leukemia Group (PALG) of a prospective, randomized, multicenter trial

Blood — Fri, 20 Apr 2007 04:00:00 +0100

In conclusion, HCL treatment with weekly 2-CdA infusions is equally effective but no safer than the standard 5-day 2-CdA protocol. (Source: Blood)

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High frequency of primary cutaneous lymphomas associated with lymphoproliferative disorders of different lineage

Annals of Hematology — Tue, 06 Mar 2007 14:08:21 +0100

Abstract??In patients suffering from primary cutaneous lymphomas, secondary malignancies of various origin may develop. However, the frequency of a second neoplasm deriving from another lymphoid lineage is still unclear and may be underestimated. We screened all our patients with primary cutaneous lymphomas from a 4-year recruitment period for a coexisting secondary lymphoproliferative disorder. The cohort comprised of a total of 82 patients with primary cutaneous lymphomas, 62 with primary cutaneous T-cell lymphoma (CTCL), 18 with primary cutaneous B-cell lymphomas, and two with CD4+/CD56+ hematodermic neoplasm/blastic lymphomas. Seven patients (8.5%) were identified with a coexisting lymphoma of a different lymphoid lineage. Four patients with S?zary syndrome (SS) suffered from systemic ...

Ribosome-lamella complex precursors in acute monocytic leukemia: a study of 6 cases.

Ultrastructural Pathology — Thu, 01 Mar 2007 05:00:00 +0100

Authors: Ru YX, Mi YC, Liu JH, Cui W, Wang HJ, Zhao SX, Jian-Xiang W The ribosome-lamella complex (RLC) is a cylindrical structure composed of annular lamella associated particles, regarded as ribosomes, around a central core, which is best known in hairy cell leukemia. RLC has been presumed to originate from aggregating rER and ribosomes. Incomplete and maturing RLC structures have been called RLC precursors (pre-RLC). The present paper investigates the various architectural aspects of pre-RLC and the ultrastructural characteristics of the blasts in 6 cases of acute monocytic leukemia (M5) in which these structures occur. Blasts bearing pre-RLC contained irregular nuclei with less heterochromatin and a prominent nucleolus, and many cytoplasmic organelles in an abundant cytoplasm. The ...

Study Examines Second Cancer Risk Among Hairy Cell Leukemia Survivors

Lymphoma / Leukemia News From Medical News Today — Sat, 10 Feb 2007 01:00:00 +0100

Survivors of a rare cancer called hairy cell leukemia are at an increased risk of Hodgkin lymphoma, non-Hodgkin lymphoma, and thyroid cancer, and at an increased risk of death from leukemia and lymphoma, according to a new study. However, the absolute risk of those second cancers is small.Hairy cell leukemia is a malignancy of a type of white blood cell called B lymphocytes. It accounts for about 2 percent of all leukemias. [click link for full article] (Source: Lymphoma / Leukemia News From Medical News Today)

Study Examines Second Cancer Risk Among Hairy Cell Leukemia Survivors

Health News from Medical News Today — Sat, 10 Feb 2007 01:00:00 +0100

Survivors of a rare cancer called hairy cell leukemia are at an increased risk of Hodgkin lymphoma, non-Hodgkin lymphoma, and thyroid cancer, and at an increased risk of death from leukemia and lymphoma, according to a new study. However, the absolute risk of those second cancers is small.Hairy cell leukemia is a malignancy of a type of white blood cell called B lymphocytes. [click link for full article] (Source: Health News from Medical News Today)

Second Cancer Incidence and Cause-Specific Mortality Among 3104 Patients With Hairy Cell Leukemia: A Population-Based Study

JNCI — Tue, 06 Feb 2007 07:00:00 +0100

Conclusions Patients with hairy cell leukemia are at increased risk of Hodgkin lymphoma, non-Hodgkin lymphoma, and thyroid cancer. The decrease in lung cancer incidence and smoking-associated vascular mortality may reflect an inverse association of tobacco use with hairy cell leukemia. Future studies should address the roles of immunologic impairment inherent to hairy cell leukemia, treatment modalities, and other factors as codeterminants of morbidity and mortality in hairy cell leukemia survivors. (Source: JNCI)

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Central Role of Protein Kinase C in Constitutive Activation of ERK1/2 and Rac1 in the Malignant Cells of Hairy Cell Leukemia

American Journal of Pathology — Wed, 31 Jan 2007 16:35:17 +0100

(Source: American Journal of Pathology)

Presence of the anti-leukemic nucleotide analog, 2-chloro-2'-deoxyadenosine-5'-monophosphate, in a promoter sequence alters DNA binding of TATA-binding protein (TBP).

Archives of Biochemistry and Biophysics — Wed, 24 Jan 2007 05:00:00 +0100

Authors: Hartman WR, Walters DE, Hentosh P 2-Chlorodeoxyadenosine (CldAdo, Cladribine), a nucleoside analog used in the treatment of hairy cell leukemia, is phosphorylated and incorporated into DNA, but is not an absolute chain terminator. We hypothesized that the presence of a chlorine molecule projecting into the DNA minor groove would affect DNA:protein-binding interactions. Here, we investigated recognition of and binding to double-stranded CldAMP-substituted TATA promoter sequences by human TATA-binding protein (TBP) using mobility shift assays. Depending on the site, CldAMP in place of dAMP within a TATA sequence decreased in vitro TBP binding by approximately 30% to 55% compared to control sites. When bound to a CldAMP-substituted TATA box, however, the TBP complex was more resi...

Hairy cell leukaemia presenting with ascites, pleural effusion and increased CA 125 serum level.

The Netherlands Journal of Medicine — Mon, 01 Jan 2007 05:00:00 +0100

Authors: Dinçol G, Do 287 Semi An O, Küçükkaya RD, Gül E, Kahraman R, A 287 Semi An M The body cavities are rarely involved in hairy cell leukaemia. Here we report a patient who had pancytopenia, hepatosplenomegaly, massive haemorrhagic ascites, pleural effusion at the left hemithorax and increased CA 125 serum level at the time of initial diagnosis. Laparoscopy showed multiple nodular white, opaque lesions on the omentum and on the parietal peritoneum. Laparoscopic biopsy of these lesions, and a bone marrow biopsy revealed a diffuse cellular infiltrate of tartrate-resistant acid phosphatase staining mononuclear cells. These mononuclear cells with irregular cytoplasmic protrusions were also found in the peripheral blood, in the ascites fluid and in the pleural ...

Possible Pentostatin-Induced Symptomatic Hyponatremia

Pharmacotherapy: Official Journal of the American College of Clinical Pharmacy — Thu, 28 Dec 2006 07:00:00 +0100

Pharmacotherapy 27(1): 164-169 Pentostatin is an adenosine deaminase inhibitor used in the treatment of hairy cell leukemia and T-cell lymphomas. A 27-year-old man with refractory cutaneous T-cell lymphoma developed severe hyponatremia 3 days after completing his first cycle of pentostatin therapy. Shortly after hospital admission, he became disoriented and was admitted to the medical intensive care unit and treated with a combination of hypertonic saline, intravenous diuretics, and fluid restriction to reestablish normal sodium homeostasis. The mechanism by which pentostatin may have induced hyponatremia in this patient is unknown; clinical and laboratory findings represented both extrarenal sodium loss and syndrome of inappropriate antidiuretic hormone. Clinicians should be aware of the ...

Multiple opportunistic infections after high-dose steroid therapy for giant cell arteritis in a patient previously treated with a purine analog.

Herpes — Tue, 12 Dec 2006 10:33:02 +0100

We present the case of a 74-y-old HIV-negative female who suffered simultaneously from multiple opportunistic infections and a Klebsiella pneumoniae sepsis during high-dose steroids for giant cell arteritis. The patient was treated with a purine analog due to hairy cell leukaemia 10 y previously. Purine analog therapy can lead to long lasting defects in cell-mediated immunity. In these patients, treatment with steroids should be closely monitored with CD4 counts. PMID: 17008240 [PubMed - indexed for MEDLINE] (Source: Herpes)

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Characterization of the B Cell Epitopes Associated with a Truncated Form of Pseudomonas Exotoxin (PE38) Used to Make Immunotoxins for the Treatment of Cancer Patients.

Journal of Immunology — Tue, 05 Dec 2006 18:25:02 +0100

In this study, we analyze the murine Ab response as a model to study the B cell epitopes associated with PE38. Sixty distinct mAbs to PE38 were characterized. Mutual competitive binding of the mAbs indicated the presence of 7 major epitope groups and 13 subgroups. The competition pattern indicated that the epitopes are discrete and could not be reproduced using a computer simulation program that created epitopes out of random surface residues on PE38. Using sera from immunotoxin-treated patients, the formation of human Abs to each of the topographical epitopes was demonstrated. One epitope subgroup, E1a, was identified as the principal neutralizing epitope. The location of each epitope on PE38 was determined by preparing 41 mutants of PE38 in which bulky surface residues were mutated to ei...

Severe Reactivation of Hepatitis B Virus Infection in a Patient with Hairy Cell Leukemia: Should Lamivudine Prophylaxis be Recommended to HBsAg-Negative, Anti-HBc-Positive Patients?

Infection — Wed, 11 Oct 2006 19:21:55 +0100

We present a male HBsAg-negative, anti-HBc-positive patient with Hairy Cell Leukemia who developed acute B hepatitis more than 1 year after the last course of 2-CdA and 6 months after splenectomy, while the patient was receiving therapy with αIFNr. The acute B hepatitis promptly responded to lamivudine therapy followed by viral clearance. Content TypeJournal Article JournalInfectionOnline ISSN 1439-0973Print ISSN 0300-8126 Journal VolumeVolume 34 Journal IssueVolume 34, Number 5 / October, 2006 (Source: Infection)

Hairy cell leukemia: towards a curative strategy.

Hematology/Oncology Clinics of North America — Sun, 24 Sep 2006 07:27:02 +0100

Authors: Gidron A, Tallman MS PMID: 16990114 [PubMed - in process] (Source: Hematology/Oncology Clinics of North America)

Immunotoxins in the treatment of refractory hairy cell leukemia.

Hematology/Oncology Clinics of North America — Sun, 24 Sep 2006 07:27:02 +0100

Authors: Kreitman RJ, Pastan I PMID: 16990113 [PubMed - in process] (Source: Hematology/Oncology Clinics of North America)

Monoclonal antibody therapy for hairy cell leukemia.

Hematology/Oncology Clinics of North America — Sun, 24 Sep 2006 07:27:02 +0100

Authors: Thomas DA, Ravandi F, Kantarjian H PMID: 16990112 [PubMed - in process] (Source: Hematology/Oncology Clinics of North America)

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Cladribine in Hairy Cell Leukemia.

Hematology/Oncology Clinics of North America — Sun, 24 Sep 2006 07:27:02 +0100

Authors: Belani R, Saven A PMID: 16990111 [PubMed - as supplied by publisher] (Source: Hematology/Oncology Clinics of North America)

Pentostatin: impact on outcome in hairy cell leukemia.

Hematology/Oncology Clinics of North America — Sun, 24 Sep 2006 07:27:02 +0100

Authors: Grever MR PMID: 16990110 [PubMed - in process] (Source: Hematology/Oncology Clinics of North America)

Purine analogues: rationale for development, mechanisms of action, and pharmacokinetics in hairy cell leukemia.

Hematology/Oncology Clinics of North America — Sun, 24 Sep 2006 07:27:02 +0100

Authors: Juliusson G, Liliemark J PMID: 16990109 [PubMed - in process] (Source: Hematology/Oncology Clinics of North America)

Splenectomy, interferon, and treatments of historical interest in hairy cell leukemia.

Hematology/Oncology Clinics of North America — Sun, 24 Sep 2006 07:27:02 +0100

Authors: Habermann TM PMID: 16990108 [PubMed - in process] (Source: Hematology/Oncology Clinics of North America)

Clinical presentations and complications of hairy cell leukemia.

Hematology/Oncology Clinics of North America — Sun, 24 Sep 2006 07:27:02 +0100

Authors: Hoffman MA PMID: 16990107 [PubMed - in process] (Source: Hematology/Oncology Clinics of North America)

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Immunophenotyping and differential diagnosis of hairy cell leukemia.

Hematology/Oncology Clinics of North America — Sun, 24 Sep 2006 07:27:02 +0100

Authors: Matutes E PMID: 16990106 [PubMed - in process] (Source: Hematology/Oncology Clinics of North America)

Hairy cell leukemia: diagnostic pathology.

Hematology/Oncology Clinics of North America — Sun, 24 Sep 2006 07:27:02 +0100

Authors: Sharpe RW, Bethel KJ PMID: 16990105 [PubMed - in process] (Source: Hematology/Oncology Clinics of North America)

Detection of CD5 in B-cell chronic lymphoproliferative diseases by flow cytometry: a strong expression in B-cell chronic lymphocytic leukemia.

Acta Cirurgica Brasileira — Thu, 14 Sep 2006 22:53:04 +0100

CONCLUSION: Our date demonstrated that CD5 expression can help distinguish among B-CLL from HCL and B-PLL, but is similar expressed in MCL. PMID: 16186976 [PubMed - as supplied by publisher] (Source: Acta Cirurgica Brasileira)

Rituximab-induced haemorrhagic thrombocytopenia in a patient with hairy cell leukaemia

British Journal of Haematology — Mon, 11 Sep 2006 10:54:00 +0100

British Journal of Haematology Volume 0, Issue 0, Page ???-???. (Source: British Journal of Haematology)

Immunotoxins for Targeted Cancer Therapy

The AAPS Journal — Fri, 18 Aug 2006 22:18:02 +0100

Immunotoxins are proteins that contain a toxin along with an antibody or growth factor that binds specifically to target cells. Nearly all protein toxins work by enzymatically inhibiting protein synthesis. For the immunotoxin to work, it must bind to and be internalized by the target cells, and the enzymatic fragment of the toxin must translocate to the cytosol. Once in the cytosol, 1 molecule is capable of killing a cell, making immunotoxins some of the most potent killing agents. Various plant and bacterial toxins have been genetically fused or chemically conjugated to ligands that bind to cancer cells. Among the most active clinically are those that bind to hematologic tumors. At present, only 1 agent, which contains human interleukin-2 and truncated diphtheria toxin, is approved for us...

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A population-based study of hairy cell leukemia in Israel

European Journal of Haematology — Tue, 08 Aug 2006 14:56:23 +0100

European Journal of Haematology Volume 0, Issue 0, Page ???-???. (Source: European Journal of Haematology)

Current treatment options in hairy cell leukemia and hairy cell leukemia variant.

Cancer Treatment Reviews — Tue, 01 Aug 2006 04:00:00 +0100

Authors: Robak T Hairy cell leukemia (HCL) is a chronic B-cell lymphoproliferative disorder characterized by splenomegaly, pancytopenia and circulating lymphocytes displaying prominent cytoplasmic projections. HCL has usually an indolent course and the patients with asymptomatic disease do not require therapy. Treatment of progressive symptomatic HCL includes a variety of pharmacological approaches such as interferon-alpha (IFN-alpha), pentostatin (DCF) and cladribine (2-CdA), which have significantly improved the disease prognosis. 2-CdA and DCF seem to induce a similar high response rate and a long overall survival. They are also active in relapsed patients. More recently high activity of anti-CD20 monoclonal antibody (rituximab) and anti-CD25 (LMB-2) and anti-CD22 (BL-22) immunotoxi...

Chronic Lymphoid Leukemias and Plasma Cell Disorders: Hairy-Cell Leukemia

Medscape Hematology-Oncology Headlines — Thu, 08 Jun 2006 06:00:00 +0100

HCL is an uncommon B cell malignancy that occurs in about 500 new patients each year in the United States.ACP Medicine Reference (Source: Medscape Hematology-Oncology Headlines)

Chronic Lymphoid Leukemias and Plasma Cell Disorders: Introduction

Medscape Hematology-Oncology Headlines — Thu, 08 Jun 2006 06:00:00 +0100

The chronic lymphoid leukemias are a group of relatively indolent clonal lymphoid disorders, primarily of B cell lineage, that includes chronic lymphocytic leukemia (CLL), prolymphocytic leukemia (PLL), hairy-cell leukemia (HCL), and the leukemic phase of non-Hodgkin lymphoma (NHL).ACP Medicine Reference (Source: Medscape Hematology-Oncology Headlines)

Eradication of minimal residual disease in hairy cell leukemia

Blood — Mon, 05 Jun 2006 06:00:00 +0100

Although the nucleoside analogs cladribine and pentostatin produce high response rates in patients with hairy cell leukemia (HCL), a significant number of patients eventually relapse. Several studies have demonstrated that patients with complete remission (CR) have a longer disease-free survival. Therefore, strategies to improve on the initial response to nucleoside analog therapy are likely to be beneficial, at least for a proportion of patients. We have treated 13 patients with newly diagnosed HCL (n = 11) or after failure of one prior chemotherapy (n = 2) with cladribine (5.6 mg/m2 given intravenously over 2 hours daily for 5 days) followed by 8 weekly doses of rituximab (375 mg/m2). All patients achieved a CR and minimal residual disease (MRD) assessed by consensus primer polymerase ch...

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Evolving concepts in the pathogenesis of hairy-cell leukaemia

Nature Reviews Cancer — Mon, 29 May 2006 06:00:00 +0100

(Source: Nature Reviews Cancer)

Role of interferon-alpha administration after 2-deoxycoformycin in the treatment of hairy cell leukemia patients

European Journal of Haematology — Wed, 26 Apr 2006 11:36:15 +0100

European Journal of Haematology Volume 0, Issue 0 (Source: European Journal of Haematology)

BL22 and lymphoid malignancies.

Best Practice & Research. Clinical Haematology — Sun, 01 Jan 2006 07:00:00 +0100

Authors: Kreitman RJ, Pastan I BL22 is a recombinant immunotoxin containing a truncated form of the bacterial toxin Pseudomonas exotoxin A attached to an Fv fragment of an anti-CD22 monoclonal antibody. Its mechanism of action involves binding to CD22, being internalized into the target cell by endocytosis, being processed to generate a free toxin fragment which is translocated into the cytoplasm, and finally induction of cell death by catalytic inactivation of elongation factor 2. In phase-I testing BL22 was very active in chemoresistant hairy-cell leukemia (HCL), with 19 (61%) of 31 patients achieving complete remission (CR). The low blood counts (cytopenias) which are characteristic of HCL improved in all complete and partial responders. Dose-limiting toxicity in HCL was due to a re...

Recent clinical trials of cladribine in hematological malignancies and autoimmune disorders.

Reviews on Recent Clinical Trials — Sun, 01 Jan 2006 05:00:00 +0100

Authors: Robak T, Wierzbowska A, Robak E The purine nucleoside analog - cladribine (2-chlorodeoxyadenosine, 2-CdA) is a cytotoxic agent with high activity in lymphoid and myeloid malignancies. It is also an effective drug in some autoimmune disorders. 2-CdA is usually administered intravenously in continuous or 2-hour infusion. Recently however, new formulation of this agent has been developed for subcutaneous and oral administration. 2-CdA is widely established as first line standard treatment for hairy cell leukemia. Moreover several clinical trials have demonstrated that this agent, used alone or in combination with other cytotoxic drugs, showed good efficacy and acceptable toxicity profile in the treatment of chronic lymphocytic leukemia, Waldenström macroglobulinemia, low-gra...

Infections associated with purine analogs and monoclonal antibodies.

Blood Reviews — Thu, 01 Sep 2005 06:00:00 +0100

Authors: Ravandi F, O'Brien S Nucleoside analogs and monoclonal antibodies are commonly used to treat lymphoproliferative disorders and have become established as the treatment of choice in chronic lymphocytic leukemia, hairy cell leukemia, and follicular lymphomas, as well as a number of other malignant lymphoid neoplasms. When used in standard doses, these agents have a low incidence of extramedullary side effects resulting in their inclusion in a number of combination regimens. The most important complications associated with these drugs are myelosuppression, immunosuppression and infections. This is further accentuated when they are used in combination with other drugs such as alkylating agents. Several investigators have attempted to delineate the risk factors predicting the risk ...

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