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This page shows you the latest news and research items in this category.

Nonsense mutation in HLA‐B*40:02 in a case with acquired aplastic anaemia: a possible origin of clonal escape from autoimmune insult
(Source: British Journal of Haematology)
Source: British Journal of Haematology - May 24, 2013 Category: Hematology Authors: Tomoo Osumi, Masashi Miharu, Hiroh Saji, Yasushi Kusunoki, Hiroto Kojima, Junko Nakamura, Hiroyuki Shimada Tags: Correspondence Source Type: research

Cyclosporin combined with levamisole for refractory or relapsed severe aplastic anaemia
(Source: British Journal of Haematology)
Source: British Journal of Haematology - May 23, 2013 Category: Hematology Authors: Yingqi Shao, Xingxin Li, Jun Shi, Meili Ge, Jinbo Huang, Zhendong Huang, Jing Zhang, Neng Nie, Yizhou Zheng Tags: correspondence Source Type: research

Interferon-{gamma} impairs proliferation of hematopoietic stem cells in mice
Balancing the processes of hematopoietic stem cell (HSC) differentiation and self-renewal is critical for maintaining a lifelong supply of blood cells. The bone marrow (BM) produces a stable output of newly generated cells, but immunologic stress conditions inducing leukopenia increase the demand for peripheral blood cell supply. Here we demonstrate that the proinflammatory cytokine interferon- (IFN-) impairs maintenance of HSCs by directly reducing their proliferative capacity and that IFN- impairs restoration of HSC numbers upon viral infection. We show that IFN- reduces thrombopoietin (TPO)-mediated phosphorylation of s...
Source: Blood - May 2, 2013 Category: Hematology Authors: de Bruin, A. M., Demirel, O., Hooibrink, B., Brandts, C. H., Nolte, M. A. Tags: Hematopoiesis and Stem Cells Source Type: research

Interpreting elevated fetal hemoglobin in pathology and health at the basic laboratory level: new and known γ‐ gene mutations associated with hereditary persistence of fetal hemoglobin
We report two novel mutations found on the promoter of the Aγ gene and summarize all common and rare determinants associated with hereditary persistence of fetal hemoglobin (HPFH) described thus far. Hematological and molecular analysis of the Aγ globin gene in two cases of HPFH. Comparison of the novel cases with all those described in the literature. We have found two novel mutations in three Italian patients with HbF values between 5.9% and 6.5% without an elevated HbA2 and with normal hemoglobin parameters. In two probands (mother and son), a −197 C>T transition was observed, while in a single individual, a −1...
Source: Clinical and Laboratory Haematology - May 1, 2013 Category: Hematology Authors: A. Amato, M. P. Cappabianca, M. Perri, I. Zaghis, P. Grisanti, D. Ponzini, P. Di Biagio Tags: Review Source Type: research

Bone marrow failure – causes and complications
Abstract: Aplastic anaemia (AA), a rare but serious form of bone marrow failure (BMF), is characterized by pancytopenia with a hypocellular bone marrow. The pathophysiology of acquired AA is predominantly immunologically mediated with damage to haematopoietic stem cells by autoreactive lymphocytes. The aberrant immune response can be triggered by environmental triggers, such as drugs, toxins, chemicals and viral infections, especially following seronegative hepatitis. Careful medical and family history, physical examination and investigations are required to rule out rarer inherited causes of bone marrow failures, and also...
Source: Medicine - May 1, 2013 Category: Internal Medicine Authors: Austin G. Kulasekararaj, Ghulam J. Mufti, Judith C.W. Marsh Tags: Marrow disorders Source Type: research

Management of immune cytopenias in patients with systemic lupus erythematosus - Old and new.
This article summarizes immune cytopenias seen in patients with SLE, and it also discusses management of these cytopenias. PMID: 23462431 [PubMed - in process] (Source: Autoimmunity Reviews)
Source: Autoimmunity Reviews - May 1, 2013 Category: Allergy & Immunology Authors: Newman K, Owlia MB, El-Hemaidi I, Akhtari M Tags: Autoimmun Rev Source Type: research

Population pharmacokinetics of ciclosporin in Chinese children with aplastic anemia: effects of weight, renal function and stanozolol administration.
Conclusion:Our model could be used to optimize the ciclosporin dosing regimen in Chinese children with aplastic anemia. PMID: 23624757 [PubMed - as supplied by publisher] (Source: Acta Pharmacologica Sinica)
Source: Acta Pharmacologica Sinica - April 29, 2013 Category: Drugs & Pharmacology Authors: Ni SQ, Zhao W, Wang J, Zeng S, Chen SQ, Jacqz-Aigrain E, Zhao ZY Tags: Acta Pharmacol Sin Source Type: research

Congenital Amegakaryocytic Thrombocytopenia (CAMT) Presenting as Severe Pancytopenia in the First Month of Life
This report emphasises the importance of considering CAMT in the differential diagnosis of congenital aplastic anaemia or idiopathic aplastic anaemia in babies. Pediatr Blood Cancer 2013;9999:XX–XX. © 2013 Wiley Periodicals, Inc. (Source: Pediatric Blood and Cancer)
Source: Pediatric Blood and Cancer - April 26, 2013 Category: Cancer & Oncology Authors: Michael T. Stoddart, Philip Connor, Manuela Germeshausen, Matthias Ballmaier, Colin G. Steward Tags: Brief Report Source Type: research

Defective telomere elongation and hematopoiesis from telomerase-mutant aplastic anemia iPSCs
Critically short telomeres activate p53-mediated apoptosis, resulting in organ failure and leading to malignant transformation. Mutations in genes responsible for telomere maintenance are linked to a number of human diseases. We derived induced pluripotent stem cells (iPSCs) from 4 patients with aplastic anemia or hypocellular bone marrow carrying heterozygous mutations in the telomerase reverse transcriptase (TERT) or the telomerase RNA component (TERC) telomerase genes. Both mutant and control iPSCs upregulated TERT and TERC expression compared with parental fibroblasts, but mutant iPSCs elongated telomeres at a lower ra...
Source: Journal of Clinical Investigation - April 19, 2013 Category: Biomedical Science Authors: Thomas Winkler, So Gun Hong, Jake E. Decker, Mary J. Morgan, Chuanfeng Wu, William M. Hughes V, Yanqin Yang, Danny Wangsa, Hesed M. Padilla-Nash, Thomas Ried, Neal S. Young, Cynthia E. Dunbar, Rodrigo T. Calado Source Type: research

Frequent Mutations in SH2D1A (XLP) in Males Presenting With High‐Grade Mature B‐Cell Neoplasms
Abstract X‐linked lymphoproliferative syndrome (XLP) is caused by mutations in SH2D1A, and is associated with overwhelming infectious mononucleosis, aplastic anemia, hypogammaglobulinemia, and B‐cell lymphomas. However, the frequency of SH2D1A mutations in males who present with B NHL is unknown. Five cases of XLP were diagnosed among 158 males presenting with B NHL (approximately 3.2%). Four of the patients had two episodes of B NHL and one had a single episode of B NHL followed by aggressive infectious mononucleosis. Prospective screening for XLP in males with B‐cell lymphoma at the time of initial diagnosis should...
Source: Pediatric Blood and Cancer - April 17, 2013 Category: Cancer & Oncology Authors: J.T. Sandlund, S.A. Shurtleff, M. Onciu, E. Horwitz, W. Leung, V. Howard, R. Rencher, M.E. Conley Tags: Brief Report Source Type: research

Reduced dose cyclophosphamide, fludarabine and antithymocyte globulin for sibling and unrelated transplant of children with severe and very severe aplastic anemia
Abstract We evaluated the results of a novel conditioning regimen of reduced dose cyclophosphamide (Cy, 25 mg/kg for four days), fludarabine (Flu, 30 mg/m2 for four days), and rabbit ATG (2.5 mg/kg for three days) for allogeneic transplant of children with SAA, implemented since January 2009. Overall, 23 patients were treated with this regimen (16 male, seven female), including 10 diagnosed with VSAA. Donors included eight‐MSD and 15 UD (five‐matched UD, and 10 mismatched UD). All patients showed neutrophil and platelet engraftment. Cumulative incidence of acute (grade 2 or above) and chronic GVHD was 26.1% and 8.7%...
Source: Pediatric Transplantation - April 3, 2013 Category: Transplant Surgery Authors: Nack‐Gyun Chung, Jae Wook Lee, Pil‐Sang Jang, Dae‐Chul Jeong, Bin Cho, Hack‐Ki Kim Tags: Original Article Source Type: research

Immune‐mediated cytopenias in human immunodeficiency virus: the first reported case of idiopathic aplastic anaemia successfully treated with immunosuppression
We report here the first case of a HIV‐infected patient who developed severe idiopathic aplastic anaemia, and who was safely and effectively treated with anti‐thymocyte globulin and cyclosporin. We briefly review immune‐mediated cytopenias in HIV, including their frequency, pathophysiology and management strategies. (Source: Internal Medicine Journal)
Source: Internal Medicine Journal - April 1, 2013 Category: Internal Medicine Authors: G. Hapgood, J. F. Hoy, C. O. Morrissey, S. M. Jane Tags: Brief Communication Source Type: research

Successful autologous cord blood transplantation in a child with acquired severe aplastic anemia
We describe a nine‐yr‐old male with SAA treated successfully with an autologous cord blood transplant following immunoablative chemotherapy. With the increasing number of people cryopreserving autologous cord blood, the use of autologous cord blood in the treatment of SAA might be considered as initial therapy. This case serves to discuss approaches to preparative therapy as well as the potential complications in this growing cohort of patients. (Source: Pediatric Transplantation)
Source: Pediatric Transplantation - March 7, 2013 Category: Transplant Surgery Authors: David Buchbinder, Loah Hsieh, Geetha Puthenveetil, Amit Soni, Jill Stites, Van Huynh, Ivan Kirov, Steve Neudorf, Elyssa Rubin, Leonard Sender, Lilibeth Torno, David Margolis, Richard Childs, Theodore Moore, Diane Nugent Tags: Case Report Source Type: research

Adefovir Is Effective to Promote Development of Immunity to Donor Origin Hepatitis B Virus in an Allogeneic Transplant Recipient: A Case Report
In conclusion adefovir was effective to treat lamivudine-resistant HBV infection in an allogeneic HCT recipient. (Source: Transplantation Proceedings)
Source: Transplantation Proceedings - March 1, 2013 Category: Transplant Surgery Authors: D.G. Yaşar, E. Suyanı, S. Özenirler, G.T. Sucak Tags: Case Reports in Transplantation Source Type: research

Cytogenetics in paediatric aplastic anaemia: Does it give an insight on the evolution of the disease?
PMID: 23640551 [PubMed - in process] (Source: The Indian Journal of Medical Research)
Source: The Indian Journal of Medical Research - March 1, 2013 Category: Biomedical Science Authors: Ghosh K Tags: Indian J Med Res Source Type: research

Cytogenetic profile of aplastic anaemia in Indian children.
We report here cytogenetic studies on paediatric patients with acquired aplastic anaemia seen in a tertiary care hospital in north India. Methods: Patients (n=71, age 4-14 yr) were diagnosed according to the guidelines of International Agranulocytosis and Aplastic Anaemia Study. Conventional cytogenetics with Giemsa Trypsin Giemsa (GTG) banding was performed. Karyotyping was done according to the International System for Human Cytogenetics Nomenclature (ISCN). Results: Of the 71 patients, 42 had successful karyotyping where median age was 9 yr; of these 42, 27 (64.3%) patients had severe, nine (21.4%) had very severe and s...
Source: The Indian Journal of Medical Research - March 1, 2013 Category: Biomedical Science Authors: Gupta V, Kumar A, Saini I, Saxena AK Tags: Indian J Med Res Source Type: research

In vivo protection of activated Tyr22‐DHFR gene‐modified canine T lymphocytes from methotrexate
ConclusionsThese findings have implications for clinical translation of MTX‐resistant T cells to facilitate engraftment of allogeneic cells following nonmyeloablative conditioning and minimize the risk of rejection. In summary, Tyr22‐DHFR expression in T lymphocytes provides chemoprotection from MTX‐mediated elimination in the context of immune activation in vivo. This article is protected by copyright. All rights reserved. (Source: The Journal of Gene Medicine)
Source: The Journal of Gene Medicine - March 1, 2013 Category: Genetics & Stem Cells Authors: Jennifer L. Gori, Brian C. Beard, Nathaniel P. Williams, Christina Ironside, Debra Swanson, R. Scott McIvor, H. P. Kiem Tags: Research Article Source Type: research

Less could be better: The case for reducing the dose of cyclophosphamide for children undergoing allogeneic stem cell transplant for severe aplastic anemia
(Source: Pediatric Transplantation)
Source: Pediatric Transplantation - March 1, 2013 Category: Transplant Surgery Authors: Ayad A. Hussein, Haydar Frangoul Tags: Editorial Source Type: research

Imatinib: Aplastic anaemia: case report
(Source: Reactions)
Source: Reactions - February 24, 2013 Category: Drugs & Pharmacology Tags: Short communication Source Type: research

Low frequency clonal mutations recoverable by deep sequencing in patients with aplastic anemia
ntin & D M Weinstock (Source: Leukemia)
Source: Leukemia - February 22, 2013 Category: Hematology Authors: A A LaneO OdejideN KoppS KimA YodaR ErlichN WagleG A AbelS J RodigJ H AntinD M Weinstock Source Type: research

Risk factors influencing mortality related to Stenotrophomonas maltophilia infection in hematology-oncology patients.
The objective of this study was to analyze the clinical characteristics and outcomes of cancer and hemopathic patients with S. maltophilia infection and assess the factors influencing the mortality. The microbiology laboratory records of Erciyes University, Faculty of Medicine Hospital were reviewed to retrospectively identify patients with S. maltophilia infection between January 2007 and June 2011. A total of 38 patients (25 male, 13 female) were eligible for the study. The median age of the patients was 53 years. The underlying disease was hematological malignancy and disorders in 76.3 % (29 cases), solid tumors in 15...
Source: International Journal of Hematology - February 22, 2013 Category: Hematology Authors: Demiraslan H, Sevim M, Pala C, Durmaz S, Berk V, Kaynar L, Metan G Tags: Int J Hematol Source Type: research

Expansion of a liver‐infiltrating cytotoxic T‐lymphocyte clone in concert with the development of hepatitis‐associated aplastic anaemia
(Source: British Journal of Haematology)
Source: British Journal of Haematology - February 19, 2013 Category: Hematology Authors: Yasuhiro Ikawa, Ryosei Nishimura, Rie Kuroda, Shintaro Mase, Raita Araki, Hideaki Maeba, Taizo Wada, Tomoko Toma, Shoichi Koizumi, Akihiro Yachie Tags: Correspondence Source Type: research

Horsemeat investigation: latest update
On February 14 the Food Standards Agency (FSA) released a statement that it had detected the presence of phenylbutazone (bute) in horses slaughtered in the UK. They tested a total of 206 horse carcasses between January 30 and February 7 2013. Of these, eight tested positive for the drug. Of these eight: Six were sent to France and may have entered the food chain. The remaining two did not leave the slaughterhouse in the UK and have now been disposed of in accordance with EU rules. The FSA is gathering information on the six carcasses sent to France and will work with the French authorities to trace them. The offi...
Source: NHS News Feed - February 15, 2013 Category: Consumer Health News Tags: Food/diet QA articles Source Type: news

A Case of Familial Myasthenia Gravis and Azathioprine-induced Bone Marrow Toxicity in Multiple Family Members (P02.193)
CONCLUSIONS: The history of these two patients with familial myasthenia gravis and azathioprine-induced bone marrow toxicity seems to suggest there may be a genetic component that may have lead to a predisposition for both. As there are no cases previously reported in the medical literature, no known genetic connection and no information about any genetic alterations in the patients in question, a definitive conclusion about the potential link cannot be drawn.Disclosure: Dr. Marky has nothing to disclose. Dr. Freberg has nothing to disclose. Dr. Faulkner has nothing to disclose. (Source: Neurology)
Source: Neurology - February 14, 2013 Category: Neurology Authors: Marky, B., Freberg, J., Faulkner, M. Tags: P02 Myasthenia Gravis: Treatment Issues Source Type: research

Mystery meat: it's what's for dinner | GrrlScientist
There's more mystery lurking in a horsemeat burger than meets the eye (and none of it is tasty)When I was an undergraduate in college, my colleagues and I would joke about the meals served in the dorm cafeteria as containing "mystery meat". At the time, I was fresh off the racetrack, and I viewed the identity of minced meat – "hamburger" in the USA – with scepticism. Shortly thereafter, I stopped eating meat altogether.Even though I love horses and now am vegan (except one or two days per week when I give in to my lust for sheep cheese), I don't have a problem with people eating horsemeat – no more than I have a prob...
Source: Guardian Unlimited Science - February 12, 2013 Category: Science Authors: GrrlScientist Tags: Blogposts guardian.co.uk Science Source Type: news

Eating horsemeat: what are the steaks? | Henry Gee
Harder than nailing jelly to the ceiling, harder even than understanding a sermon by Dr Rowan Williams, is trying to get the general public to appreciate what is meant by 'risk'Many years ago when the world was young (okay, it was 1993) I was enjoying a leisurely lunch at CalTech in Pasadena with seismologist Dr Lucille Jones. She spent much time engaged in outreach, she explained, trying to persuade the public of California to take appropriate precautions in case of earthquakes. The problem was making sure people were well-informed without instilling mass panic. Earthquakes powerful enough to cause serious damage don't oc...
Source: Guardian Unlimited Science - February 12, 2013 Category: Science Authors: Henry Gee Tags: Blogposts Health guardian.co.uk Mathematics Food & drink Society Horsemeat scandal Life and style Food science Source Type: news

Increased plasma thrombopoietin levels in patients with myelodysplastic syndrome: a reliable marker for a benign subset of bone marrow failure.
Conclusions: Increased plasma thrombopoietin levels were associated with a favorable prognosis of bone marrow failure and could therefore represent a reliable marker for a benign subset of myelodysplastic syndrome. PMID: 23403320 [PubMed - as supplied by publisher] (Source: Haematologica)
Source: Haematologica - February 12, 2013 Category: Hematology Authors: Seiki Y, Sasaki Y, Hosokawa K, Saito C, Sugimori N, Yamazaki H, Takami A, Nakao S Tags: Haematologica Source Type: research

Official advice about horsemeat safety released
Concerns have been raised about the safety of horsemeat in the wake of the contaminated beef scandal. The Daily Mail asks, 'Is horsemeat harmful after all?', while The Guardian states that, 'Contaminated horsemeat could harm health, warns environment secretary.' Horsemeat has been confirmed to have been present in several products labelled and sold as "beef". Horsemeat in itself is not a health risk, with some commentators arguing it is actually healthier than beef as it contains less fat. The main worry is that as the horsemeat has been illegally introduced into the food chain, it might not comply with the sta...
Source: NHS News Feed - February 11, 2013 Category: Consumer Health News Tags: Food/diet QA articles Source Type: news

Eczema herpeticum with herpetic folliculitis after bone marrow transplant under prophylactic acyclovir: are patients with underlying dermatologic disorders at higher risk?
We present an unreported coexistence: eczema herpeticum (EH) with histopathological findings of herpetic folliculitis (HF) after allogeneic bone marrow transplantation (BMT). A patient with atopic dermatitis (AD) underwent allogeneic BMT for idiopathic acquired aplastic anemia. She had been receiving cyclosporine (150 mg/12 h) and acyclovir (400 mg/12 h) for 6 months. A facial rash was observed, composed of monotonous erythematous, umbilicated papulo-vesicles and papulo-crusts <4 mm in size. The histopathological study showed herpetic cytopathic changes within the epidermis that extended into the hair follicle epi...
Source: Herpes - February 6, 2013 Category: Infectious Diseases Authors: Mir-Bonafé JM, Román-Curto C, Santos-Briz A, Palacios-Álvarez I, Santos-Durán JC, Fernández-López E Tags: Transpl Infect Dis Source Type: research

Eczema herpeticum with herpetic folliculitis after bone marrow transplant under prophylactic acyclovir: are patients with underlying dermatologic disorders at higher risk?
We present an unreported coexistence: eczema herpeticum (EH) with histopathological findings of herpetic folliculitis (HF) after allogeneic bone marrow transplantation (BMT). A patient with atopic dermatitis (AD) underwent allogeneic BMT for idiopathic acquired aplastic anemia. She had been receiving cyclosporine (150 mg/12 h) and acyclovir (400 mg/12 h) for 6 months. A facial rash was observed, composed of monotonous erythematous, umbilicated papulo‐vesicles and papulo‐crusts <4 mm in size. The histopathological study showed herpetic cytopathic changes within the epidermis that extended into the hair follicle...
Source: Transplant Infectious Disease - February 6, 2013 Category: Transplant Surgery Authors: J.M. Mir‐Bonafé, C. Román‐Curto, A. Santos‐Briz, I. Palacios‐Álvarez, J.C. Santos‐Durán, E. Fernández‐López Tags: Case report Source Type: research

Excellent Outcome of Haploidentical Hematopoietic Stem Cell Transplantation in Children and Adolescents with Acquired Severe Aplastic Anemia
Abstract: We evaluated the outcome of children and adolescents with acquired severe aplastic anemia (SAA) who received haploidentical hematopoietic stem cell transplantation (HHCT) with in vitro T cell–depleted peripheral blood stem cells. Twelve patients with acquired SAA received a total of 15 HHCTs with in vitro CD3-depleted grafts between July 2009 and July 2012. Among the 12 patients, 11 achieved neutrophil engraftment at a median of 10 days (range, 9 to 13 days) after HHCT. One patient failed to achieve primary engraftment, and two experienced graft rejection soon after engraftment. All three patients who experie...
Source: Biology of Blood and Marrow Transplantation - February 4, 2013 Category: Hematology Authors: Ho Joon Im, Kyung Nam Koh, Eun Seok Choi, Seongsoo Jang, Seog Woon Kwon, Chan-Jeoung Park, Hyun-Sook Chi, Jong Jin Seo Tags: Clinical Research Source Type: research

Wang et al. Cotransplantation of Allogeneic Mesenchymal and Hematopoietic Stem Cells in Children With Aplastic Anemia. Pediatrics. 2012;129(6):e1612-e1615
(Source: PEDIATRICS)
Source: PEDIATRICS - February 1, 2013 Category: Pediatrics Tags: Office Practice Errata Source Type: research

Comparison of the efficacy of rabbit and horse antithymocyte globulin for the treatment of severe aplastic anemia in children
(Source: Blood)
Source: Blood - January 31, 2013 Category: Hematology Authors: Yoshimi, A., Niemeyer, C. M., Fuhrer, M. M., Strahm, B. Tags: CORRESPONDENCE Source Type: research

Rabbit antithymocyte globulin and cyclosporine as first-line therapy for children with acquired aplastic anemia
(Source: Blood)
Source: Blood - January 31, 2013 Category: Hematology Authors: Takahashi, Y., Muramatsu, H., Sakata, N., Hyakuna, N., Hamamoto, K., Kobayashi, R., Ito, E., Yagasaki, H., Ohara, A., Kikuchi, A., Morimoto, A., Yabe, H., Kudo, K., Watanabe, K.-i., Ohga, S., Kojima, S., on behalf of the Japan Childhood Aplastic Anemia St Tags: CORRESPONDENCE Source Type: research

Cytopenia and autoimmune diseases: A vicious cycle fueled by mTOR dysregulation in hematopoietic stem cells.
Abstract A long-standing but poorly understood defect in autoimmune diseases is dysfunction of the hematopoietic cells. Leukopenia is often associated with systemic lupus erythematous (SLE) and other autoimmune diseases. In addition, homeostatic proliferation of T cells, which is a host response to T-cell lymphopenia, has been implicated as potential cause of rheumatoid arthritis (RA) in human and experimental models of autoimmune diabetes in the NOD mice and the BB rats. Conversely, successful treatments of aplastic anemia by immune suppression suggest that the hematologic abnormality may have a root in autoimmune...
Source: Journal of Autoimmunity - January 31, 2013 Category: Allergy & Immunology Authors: Zheng P, Chang X, Lu Q, Liu Y Tags: J Autoimmun Source Type: research

The progression risk factors of children with transfusion-independent non-severe aplastic anemia.
Abstract Non-severe aplastic anemia (NSAA) in children is a rare, idiopathic condition of bone marrow insufficiency that can resolve spontaneously, persist for months or years, or progress to severe aplastic anemia (SAA). We reviewed clinical and laboratory data of patients diagnosed with transfusion-independent non-severe aplastic anemia (NSAA) from 1996 to 2009 at the Institute of Hematology and Blood Diseases Hospital, Peking Union Medical College, and analyzed the clinical course and outcomes in these patients. NSAA was defined as bone marrow cellularity <50 % and two or three cytopenias that persisted for ...
Source: International Journal of Hematology - January 30, 2013 Category: Hematology Authors: Wang S, Chen Y, Zou Y, Zheng Y, Zhu X Tags: Int J Hematol Source Type: research

Transient JAK2 V617F mutation in an aplastic anaemia patient with a paroxysmal nocturnal haemoglobinuria clone
(Source: British Journal of Haematology)
Source: British Journal of Haematology - January 30, 2013 Category: Hematology Authors: Stephen E. Langabeer, Karl Haslam, David O'Brien, Helen Enright, Maeve Leahy Tags: Correspondence Source Type: research

In vivo T‐cell depletion with alemtuzumab in allogeneic hematopoietic stem cell transplantation: Combined results of two studies on aplastic anemia and HLA‐mismatched haploidentical transplantation
Abstract We evaluated the efficacy of in vivo T‐cell depletion with alemtuzumab in two prospective studies according to the ICH‐GCP guidelines; one was for patients with aplastic anemia (AA study) and the other was for patients who were undergoing hematopoietic stem cell transplantation (HSCT) from a 2‐ or 3‐antigen‐mismatched haploidentical donor (MM study). The final dose of alemtuzumab in these studies was 0.16 mg/kg/day for 6 days. At this dose, all of the 12 and 11 patients in the AA and MM studies, respectively, achieved initial engraftment and the incidences of grade II‐IV acute GVHD were 0% and 18%. Whi...
Source: American Journal of Hematology - January 24, 2013 Category: Hematology Authors: Yoshinobu Kanda, Kumi Oshima, Shinichi Kako, Takahiro Fukuda, Naoyuki Uchida, Koichi Miyamura, Yukio Kondo, Shinji Nakao, Koji Nagafuji, Toshihiro Miyamoto, Mineo Kurokawa, Yasushi Okoshi, Shigeru Chiba, Yasuo Ohashi, Yoichi Takaue, Shuichi Taniguchi Tags: Research Article Source Type: research

[Dyskeratosis congenita: An update.]
Abstract Dyskeratosis congenita is a rare inherited bone marrow failure characterized by excessively short telomeres in highly proliferative tissues. These abnormalities are due to disturbance of the telomere maintenance machinery. The clinical presentation is characterized by skin pigmentation, nail dystrophy, and mucosal leukoplakia. All these mucocutaneous features are rare in childhood: they usually appear between 5 and 10years of age. In young children, the initial presentation can associate bone marrow failure and neurological or ocular problems: Hoyeraal-Hreidarsson and Revesz syndromes, respectively. Clinic...
Source: Archives de Pediatrie - January 23, 2013 Category: Pediatrics Authors: Mialou V, Leblanc T, Latour RP, Dalle JH, Socié G Tags: Arch Pediatr Source Type: research

LCNDG Rapid Review: Anti-thymocyte globulin (horse) for the first-line treatment of aplastic anaemia
Source: London Cancer New Drugs Group Area: Evidence > Drug Specific Reviews Immunosuppressive therapy with antithymocyte globulin (ATG) (usually with ciclosporin) has been the standard first-line treatment for patients with aplastic anaemia who are not eligible for HSCT for decades. Horse ATG (Lymphoglobuline®) was used first-line in the UK for many years but this was withdrawn from the market in 2007 - rabbit ATG (Thymoglobuline®) was then used as a substitute. However recently published data has shown that outcomes are inferior for this product compared to horse ATG and this has led to the BCSH r...
Source: NeLM - Drug Specific Reviews - January 22, 2013 Category: Drugs & Pharmacology Source Type: news

NETAG issues appraisal and recommendation on anti-thymocyte globulin (ATG) for aplastic anaemia
Source: NETAG Area: Evidence > Drug Specific Reviews The NHS North East Treatment Advisory Group (NETAG) has undertaken an appraisal of anti-thymocyte globulin (ATG) for first-line treatment of adult aplastic anaemia. The Group recommends horse ATG as first-line immunosuppressive therapy in preference to rabbit ATG for the treatment of adult aplastic anaemia, acknowledging the substantial difference in efficacy between the two (demonstrated in a single randomised study and supported by the majority of data from non-comparative studies). Recent clinical guidelines support horse ATG in preference to rabbit ATG as first...
Source: NeLM - Drug Specific Reviews - January 21, 2013 Category: Drugs & Pharmacology Source Type: news

High-dose cyclophosphamide compared with antithymocyte globulin for treatment of acquired severe aplastic anemia
A modified regimen of high-dose cyclophosphamide (CTX) plus cyclosporine (CsA) was adopted for patients with severe or very severe aplastic anemia, and the effectiveness was compared with a regimen of antithymocyte globulin (ATG) plus CsA. A total of 121 patients enrolled in this study received either CTX plus CsA (CTX group, 48 cases) or ATG plus CsA (ATG group, 73 cases). The early death rate was 4.2% in the CTX group and 8.2% in the ATG group, showing no significant difference (p = 0.312). The total response rate in the CTX and ATG groups was 54.2% and 57.5% at 3 months, 64.6% and 72.6% at 6 months, and 72.9% and 78.1% ...
Source: Experimental Hematology - January 11, 2013 Category: Hematology Authors: Fengkui Zhang, Li Zhang, Liping Jing, Kang Zhou, Huijun Wang, Guangxin Peng, Yang Li, Yuan Li, Jianping Li, Lei Ye, Lihui Shi, Huihui Fan, Xin Zhao, Yulin Chu, Yushu Hao, Jianxiang Wang Tags: Clinical Investigations Source Type: research

Cord blood transplantation in aplastic anemia
& G Socié (Source: Bone Marrow Transplantation)
Source: Bone Marrow Transplantation - January 7, 2013 Category: Hematology Authors: R Peffault de LatourV RochaG Socié Tags: cord blood transplantation aplastic anemia Source Type: research

Management of acquired aplastic anemia in children
& A A Hussein (Source: Bone Marrow Transplantation)
Source: Bone Marrow Transplantation - January 7, 2013 Category: Hematology Authors: E T KorthofA N BékássyA A Hussein Tags: acquired aplastic anemia childhood supportive care management hematopoietic SCT immunosuppressive therapy Source Type: research

Haploidentical transplantation in patients with acquired aplastic anemia
& E T Korthof (Source: Bone Marrow Transplantation)
Source: Bone Marrow Transplantation - January 7, 2013 Category: Hematology Authors: F CiceriM T Lupo-StanghelliniE T Korthof Tags: aplastic anemia haploidentical ATG Source Type: research

A Young Adult with Aplastic Anemia and Gray Hair [Clinical Case Study]
(Source: Clinical Chemistry)
Source: Clinical Chemistry - January 2, 2013 Category: Chemistry Authors: Guinan, E. C., Agarwal, S. Tags: Other Areas of Clinical Chemistry Clinical Case Study Source Type: research

Mucormycosis-associated intracranial hemorrhage
We report a patient that presented with aplastic anemia, subsequently complicated by systemic mucormycosis, which generated reactive plasmacytosis, and developed intracranial infarction and hemorrhage. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - January 1, 2013 Category: Hematology Tags: Case Reports Source Type: research

Engraftment of heavily transfused patients with severe aplastic anemia with a fludarabine‐based regimen
In conclusion, this conditioning indicated well toleration, mild toxicity, durable engraftment, excellent survival as well as less cost. Its application might shed new light on SAA at high risk of graft rejection in resource‐limited countries. (Source: Clinical Transplantation)
Source: Clinical Transplantation - January 1, 2013 Category: Transplant Surgery Authors: San‐Bin Wang, Li Li, Xin‐Hua Pan, Deng‐Ming Hu, Li‐Hui Peng, Lin Liu, Zheng‐Jun Xie, Bo Yin, Xiao‐Juan Sun, Jing Yu, Yang Liang Tags: Original Article Source Type: research

Differentiation of Fanconi anemia and aplastic anemia using mitomycin C test in Tunisia.
In this study, we provide a detailed laboratory protocol for accurate assessment of FA diagnosis based on mitomycin C (MMC) test. Induced chromosomal breakage study was successful in 171 out of 205 aplastic anemia (AA) patients. According to the sensitivity of MMC at 50 ng/ml, 38 patients (22.22%) were diagnosed as affected and 132 patients (77.17%) as unaffected. Somatic mosaicism was suspected in an 11-year-old patient with a FA phenotype. Twenty-six siblings of FA patients were also evaluated and five of them (19.23%) were diagnosed as FA. From this study, a standard protocol for diagnosis of FA was developed. It is rou...
Source: Comptes Rendus Biologies - January 1, 2013 Category: Biology Authors: Talmoudi F, Kilani O, Ayed W, Ben Halim N, Mellouli F, Torjmane L, Aissaoui L, Ben Youssef Y, Kammoun L, Ben Othmane T, Bejaoui M, Ben Romdhane N, Elloumi M, Hadiji S, Hentati S, Chemkhi I, Abidli N, Guermani H, Abdelhak S, Amouri A Tags: C R Biol Source Type: research

Conditioning with Purine Analogs Leads to Good Engraftment Rates of Immunodepleted Grafts for Aplastic Anemia
Abstract: Immunodepletion with alemtuzumab is an effective strategy for preventing graft-versus-host disease after allogeneic stem cell transplantation (SCT), but it may be associated with graft failure. We tested the effectiveness of a purine analog-based reduced-intensity conditioning combination in patients undergoing allogeneic SCT for bone marrow aplasia. Patients with severe marrow aplasia who had a tissue-compatible sibling donor were conditioned with fludarabine 30 mg/m2 for 5 days and cyclophosphamide 120 mg/kg. Stem cells from HLA-identical sibling donors were mobilized with filgrastim, and the harvested blood co...
Source: Biology of Blood and Marrow Transplantation - December 27, 2012 Category: Hematology Authors: Nicolas Novitzky, Valda Thomas, Cecile du Toit Tags: Clinical Research Source Type: research