abstract：:Guidelines on hereditary spherocytosis (HS) published in 2004 (Bolton-Maggs et al, 2004) are here replaced to reflect changes in current opinion on the surgical management, (particularly the indications for concomitant splenectomy with cholecystectomy in children with mild HS, and concomitant cholecystectomy with sple...

journal_title：British journal of haematology

authors： Bolton-Maggs PH,Langer JC,Iolascon A,Tittensor P,King MJ,General Haematology Task Force of the British Committee for Standards in Haematology.

更新日期：2012-01-01 00:00:00

abstract：:Monoclonal antibody (mAb) drugs are desirable for the improvement of multiple myeloma (MM) treatment. In this study, we found for the first time that CD48 was highly expressed on MM plasma cells. In 22 out of 24 MM patients, CD48 was expressed on more than 90% of MM plasma cells at significantly higher levels than it ...

journal_title：British journal of haematology

authors： Hosen N,Ichihara H,Mugitani A,Aoyama Y,Fukuda Y,Kishida S,Matsuoka Y,Nakajima H,Kawakami M,Yamagami T,Fuji S,Tamaki H,Nakao T,Nishida S,Tsuboi A,Iida S,Hino M,Oka Y,Oji Y,Sugiyama H

更新日期：2012-01-01 00:00:00

abstract：:Data on outcome, prognostic factors, and treatment for very elderly non-Hodgkin lymphomas (NHL) is sparse. We conducted a multicentre retrospective analysis of NHL patients ≥80 years (at diagnosis) treated between 1999 and 2009. Detailed characteristics were obtained including geriatric syndromes, activities of daily ...

journal_title：British journal of haematology

authors： Nabhan C,Smith SM,Helenowski I,Ramsdale E,Parsons B,Karmali R,Feliciano J,Hanson B,Smith S,McKoy J,Larsen A,Hantel A,Gregory S,Evens AM

更新日期：2012-01-01 00:00:00

abstract：:Acquired haemophilia A (AHA) is a rare bleeding disorder characterized by the sudden generation of autoantibodies against factor VIII (FVIII) in individuals with no previous history of abnormal haemostasis. Understanding the pathogenesis of this disease has been hampered by the rarity of the patients and the difficult...

journal_title：British journal of haematology

authors： Mahendra A,Padiolleau-Lefevre S,Kaveri SV,Lacroix-Desmazes S

更新日期：2012-01-01 00:00:00

abstract：:The BRAF V600E mutation has recently been described in all cases of hairy cell leukaemia (HCL). We have developed and validated a rapid and sensitive high-resolution melting analysis (HRMA) assay that detects BRAF exon 15 mutations when hairy cells are as low as 5-10% in a sample. All 48 HCL patients were positive for...

journal_title：British journal of haematology

authors： Boyd EM,Bench AJ,van 't Veer MB,Wright P,Bloxham DM,Follows GA,Scott MA

更新日期：2011-12-01 00:00:00

abstract：:Osteonecrosis is a disabling complication in children and young adults with acute lymphoblastic leukaemia. It can affect any or multiple joints but the hip and knee are most frequently involved and a cause of long-term disability. The problem is almost exclusively that of older children and young adults of whom over 7...

journal_title：British journal of haematology

authors： Vora A

更新日期：2011-12-01 00:00:00

abstract：:Three distinct categories of marginal zone lymphomas (MZLs) are currently recognized, principally based on their site of occurrence. They are thought to represent unique entities, but the relationship of one subtype with another is poorly understood. We investigated 17 non-splenic MZLs (seven nodal, 10 extranodal) by ...

journal_title：British journal of haematology

authors： Novak U,Basso K,Pasqualucci L,Dalla-Favera R,Bhagat G

更新日期：2011-11-01 00:00:00

abstract：:B-cell clonality analysis is commonly performed by polymerase chain reaction (PCR) targeting the IGH genes although a high false-negative rate is recognized for germinal centre/post-germinal centre B-cell malignancies, especially follicular lymphoma. We assessed the diagnostic value of BIOMED-2 IGK assays and investig...

journal_title：British journal of haematology

authors： Payne K,Wright P,Grant JW,Huang Y,Hamoudi R,Bacon CM,Du MQ,Liu H

更新日期：2011-10-01 00:00:00

abstract：:Juvenile myelomonocytic leukaemia (JMML) is a unique myeloproliferative disorder of early childhood. Frequently, mutations in NRAS, KRAS, PTPN11, NF1 or CBL are found in these patients. Monosomy 7 is the most common cytogenetic aberration. To identify submicroscopic genomic copy number alterations, 20 JMML samples wer...

journal_title：British journal of haematology

authors： Ripperger T,Tauscher M,Praulich I,Pabst B,Teigler-Schlegel A,Yeoh A,Göhring G,Schlegelberger B,Flotho C,Niemeyer CM,Steinemann D

更新日期：2011-10-01 00:00:00

abstract：:This study compared pulmonary function tests (PFTs) with cardiac, pancreatic and liver iron in 76 thalassemia major (TM) patients. Restrictive lung disease was observed in 16%, hyperinflation in 32% and abnormal diffusing capacity in 3%. While no patients met Global Initiative for Chronic Lung Disease criteria for air...

journal_title：British journal of haematology

authors： Sohn EY,Noetzli LJ,Gera A,Kato R,Coates TD,Harmatz P,Keens TG,Wood JC

更新日期：2011-10-01 00:00:00

abstract：:The prevalence of obesity has increased substantially over recent years. Clinicians are increasingly being challenged with making uncertain anticoagulant dosing decisions, as the optimal dosing strategy for most anticoagulants in the obese patient population remains unknown. Research published to date suggests that th...

journal_title：British journal of haematology

authors： Patel JP,Roberts LN,Arya R

更新日期：2011-10-01 00:00:00

abstract：:Analogous to T cells, Natural Killer (NK) cells may facilitate engraftment, combat infection, and control cancer in bone marrow or haematopoietic stem cell transplantation (HSCT); however, NK cells do not cause graft-versus-host disease. Killer immunoglobulin-like receptors (KIRs) regulate NK cell function, and recent...

journal_title：British journal of haematology

authors： Leung W

更新日期：2011-10-01 00:00:00

abstract：:The translocation t(1;19)(q23;p13)/der(19)t(1;19) is a risk stratifying aberration in childhood B-cell precursor acute lymphoblastic leukaemia (BCP ALL) in the Nordic countries. We have identified 47 children/adolescents with t(1;19)/der(19)t(1;19)-positive BCP ALL treated on two successive Nordic Society of Paediatri...

journal_title：British journal of haematology

authors： Andersen MK,Autio K,Barbany G,Borgström G,Cavelier L,Golovleva I,Heim S,Heinonen K,Hovland R,Johannsson JH,Johansson B,Kjeldsen E,Nordgren A,Palmqvist L,Forestier E

更新日期：2011-10-01 00:00:00

abstract：:Novel therapies have transformed the treatment paradigm for multiple myeloma with significant improvements in survival now seen in both younger and older patients. Nonetheless, the disease is heterogeneous and high-risk patients in particular continue to have poor outcome. Moreover, the disease remains incurable. Effo...

journal_title：British journal of haematology

authors： Richardson PG,Laubach J,Mitsiades CS,Schlossman R,Hideshima T,Redman K,Chauhan D,Ghobrial IM,Munshi N,Anderson KC

更新日期：2011-09-01 00:00:00

abstract：:Distinguishing Burkitt lymphoma (BL) from B cell lymphoma, unclassifiable with features intermediate between diffuse large B-cell lymphoma (DLBCL) and BL (DLBCL/BL), and DLBCL is challenging. We propose an immunohistochemistry and fluorescent in situ hybridization (FISH) based scoring system that is employed in three ...

journal_title：British journal of haematology

authors： Naresh KN,Ibrahim HA,Lazzi S,Rince P,Onorati M,Ambrosio MR,Bilhou-Nabera C,Amen F,Reid A,Mawanda M,Calbi V,Ogwang M,Rogena E,Byakika B,Sayed S,Moshi E,Mwakigonja A,Raphael M,Magrath I,Leoncini L

更新日期：2011-09-01 00:00:00

abstract：:Sickle cell anaemia is a severe inherited blood disorder for which there is presently no curative therapy other than allogeneic haematopoietic stem cell (HSC) transplantation. This therapeutic option, however, is not available to most patients because of the lack of a matched related donor. Different genetic strategie...

journal_title：British journal of haematology

authors： Mansilla-Soto J,Rivière I,Sadelain M

更新日期：2011-09-01 00:00:00

abstract：:The integration of molecular and clinical information to tailor treatments remains an important research challenge in chronic lymphocytic leukaemia (CLL). This study aimed to identify genomic lesions associated with a poor outcome and a higher risk of histological transformation. A mono-institutional cohort of 147 cas...

journal_title：British journal of haematology

authors： Rinaldi A,Mian M,Kwee I,Rossi D,Deambrogi C,Mensah AA,Forconi F,Spina V,Cencini E,Drandi D,Ladetto M,Santachiara R,Marasca R,Gattei V,Cavalli F,Zucca E,Gaidano G,Bertoni F

更新日期：2011-09-01 00:00:00

abstract：:To date, there is a lack of long-term safety and efficacy data for iron chelation therapy in transfusion-dependent patients with sickle cell disease (SCD). To evaluate the long-term safety and efficacy of deferasirox (a once-daily oral iron chelator), patients with SCD completing a 1-year, Phase II, randomized, defero...

journal_title：British journal of haematology

authors： Vichinsky E,Bernaudin F,Forni GL,Gardner R,Hassell K,Heeney MM,Inusa B,Kutlar A,Lane P,Mathias L,Porter J,Tebbi C,Wilson F,Griffel L,Deng W,Giannone V,Coates T

更新日期：2011-08-01 00:00:00

abstract：:Chromosomal rearrangements involving the MECOM (MDS1 and EVI1 complex) locus are recurrent genetic events in myeloid leukaemia and are associated with poor prognosis. In this study, we assessed the role of MECOM locus protein EVI1 in the transcriptional regulation of microRNAs (miRNAs) involved in the leukaemic phenot...

journal_title：British journal of haematology

authors： De Weer A,Van der Meulen J,Rondou P,Taghon T,Konrad TA,De Preter K,Mestdagh P,Van Maerken T,Van Roy N,Jeison M,Yaniv I,Cauwelier B,Noens L,Poirel HA,Vandenberghe P,Lambert F,De Paepe A,Sánchez MG,Odero M,Verhasselt

更新日期：2011-08-01 00:00:00

abstract：:Novel agents have provided a new foundation for multiple myeloma therapies. When combined with other anti-myeloma agents, these compounds significantly enhance clinical efficacy. High-dose steroids are frequently used in anti-myeloma combination regimens; however, the doses employed are often poorly tolerated, especia...

journal_title：British journal of haematology

authors： Sher T,Ailawadhi S,Miller KC,Manfredi D,Wood M,Tan W,Wilding G,Czuczman MS,Hernandez-Ilizaliturri FJ,Hong F,Sood R,Soniwala S,Lawrence W,Jamshed S,Masood A,Iancu D,Lee K,Chanan-Khan A

更新日期：2011-07-01 00:00:00

abstract：:Myeloid-derived suppressor cells (MDSCs) are a heterogeneous population of immature myeloid cells at different stages of maturation that play a role in cancer tolerance and function as an immune-suppressive cell subpopulation. They utilize different mechanisms to block both innate and adaptive arms of anti-tumour immu...

journal_title：British journal of haematology

authors： Tadmor T,Attias D,Polliack A

更新日期：2011-06-01 00:00:00

abstract：:Paroxysmal nocturnal haemoglobinuria (PNH) is a serious form of acquired haemolytic anaemia with several features that make it unique, including the fact that it is caused by clonal expansion, in the context of bone marrow failure, of a haematopoietic stem cell that has a somatic mutation in a gene crucial for the syn...

journal_title：British journal of haematology

authors： Luzzatto L,Gianfaldoni G,Notaro R

更新日期：2011-06-01 00:00:00

abstract：:Aplastic anaemia (AA) is considered as an immune-mediated bone marrow failure syndrome. The mechanism is involved with a variety of immune molecules including interferon-γ (IFN-γ), tumour necrosis factor-α (TNF-α) and interleukins (ILs). IL-27 is a novel member of the IL-12 family, which mediates T cell response and e...

journal_title：British journal of haematology

authors： Li J,Zhao Q,Xing W,Feng J,Wu H,Li H,Ge M,Tian K,Li X,Zhou J,Liu B,Zhang L,Zheng Y,Han ZC

更新日期：2011-06-01 00:00:00

abstract：:Bacteria in platelet components (PC) may result in transfusion-related sepsis (TRS). Pathogen inactivation of PC with amotosalen (A-PC) can abrogate the risk of TRS and hence facilitate storage to 7 d. A randomized, controlled, double-blinded trial to evaluate the efficacy and safety of A-PC stored for 6-7 d was condu...

journal_title：British journal of haematology

authors： Lozano M,Knutson F,Tardivel R,Cid J,Maymó RM,Löf H,Roddie H,Pelly J,Docherty A,Sherman C,Lin L,Propst M,Corash L,Prowse C

更新日期：2011-05-01 00:00:00

abstract：:The peripheral T-cell neoplasms are a biologically and clinically heterogeneous group of rare disorders that result from clonal proliferation of mature post-thymic lymphocytes. Natural killer (NK) cell neoplasms are included in this group. The World Health Organization classification of haemopoietic malignancies has d...

journal_title：British journal of haematology

authors： Dearden CE,Johnson R,Pettengell R,Devereux S,Cwynarski K,Whittaker S,McMillan A,British Committee for Standards in Haematology.

更新日期：2011-05-01 00:00:00

abstract：:This article reviews the use of aberrant antigen expression detected by flow cytometry in the diagnosis and clinical handling of acute myeloid leukaemia (AML) and the myelodysplastic syndromes (MDS). Such aberrancies offer a valuable tool for the proper classification of these myeloid malignancies according the World ...

journal_title：British journal of haematology

authors： Ossenkoppele GJ,van de Loosdrecht AA,Schuurhuis GJ

更新日期：2011-05-01 00:00:00

abstract：:The myelodysplastic syndromes (MDS) display both haematological and biological heterogeneity with variable leukaemia potential. MicroRNAs play an important role in tumour suppression and the regulation of self-renewal and differentiation of haematopoietic progenitors. Using a microarray platform, we evaluated microRNA...

journal_title：British journal of haematology

authors： Sokol L,Caceres G,Volinia S,Alder H,Nuovo GJ,Liu CG,McGraw K,Clark JA,Sigua CA,Chen DT,Moscinski L,Croce CM,List AF

更新日期：2011-04-01 00:00:00

abstract：:There is little information published comparing peripheral blood stem cells (PBSC) with bone marrow (BM) as the stem cell source in the long-term outcome in recipients of T-cell depleted (TCD) unrelated donor (UD) transplants. We present retrospective outcome data on 306 recipients of myeloablative, human leucocyte an...

journal_title：British journal of haematology

authors： Shaw BE,Apperley JF,Russell NH,Craddock C,Liakopoulou E,Potter MN,Wynn R,Gibson B,Pearce RM,Kirkland K,Lee J,Madrigal JA,Cook G,Byrne JL

更新日期：2011-04-01 00:00:00

abstract：:We analysed the results of haematopoietic cell transplantation (HCT) in 30 patients aged 60-78 (median 65) years, with primary myelofibrosis or myelofibrosis evolving from antecedent polycythaemia vera or essential thrombocythaemia. Donors were human leucocyte antigen (HLA)-identical siblings (N = 15) or unrelated ind...

journal_title：British journal of haematology

authors： Samuelson S,Sandmaier BM,Heslop HE,Popat U,Carrum G,Champlin RE,Storb R,Prchal JT,Gooley TA,Deeg HJ

更新日期：2011-04-01 00:00:00

abstract：:Minimal residual disease (MRD) assessment is a powerful prognostic factor for determining the risk of relapse in childhood acute lymphoblastic leukaemia (ALL). In this Swedish multi-centre study of childhood ALL diagnosed between 2002 and 2006, the MRD levels were analysed in 726 follow-up samples in 228 children usin...

journal_title：British journal of haematology

authors： Thörn I,Forestier E,Botling J,Thuresson B,Wasslavik C,Björklund E,Li A,Lindström-Eriksson E,Malec M,Grönlund E,Torikka K,Heldrup J,Abrahamsson J,Behrendtz M,Söderhäll S,Jacobsson S,Olofsson T,Porwit A,Lönnerholm G,R

更新日期：2011-03-01 00:00:00

abstract：:The prognostic role of CD20 expression and Epstein-Barr virus (EBV) positivity in post-transplant lymphoproliferative disease (PTLD) after solid organ transplantation (SOT) in paediatric patients is poorly understood. We retrospectively examined the relationship of CD20 and EBV with the time interval from SOT to PTLD ...

journal_title：British journal of haematology

authors： Orjuela MA,Alobeid B,Liu X,Siebert AL,Kott ER,Addonizio LJ,Morris E,Garvin JH,Lobritto SJ,Cairo MS

更新日期：2011-03-01 00:00:00

abstract：:Ernest Beutler was one of the preeminent haematologists of the last half of the 20th and the early 21st century. In a career that spanned six decades, his research interests included such diverse areas as red cell metabolism, blood preservation, glycolipid storage diseases, leukaemias and iron metabolism. Indeed, he w...

journal_title：British journal of haematology

authors： Beutler S,Beutler B

更新日期：2011-03-01 00:00:00

abstract：:Myeloid neoplasms derive from the pathological clonal expansion of an abnormal stem cell and span a diverse spectrum of phenotypes including acute myeloid leukaemia (AML), myeloproliferative neoplasms (MPN) and myelodysplastic syndromes (MDS). Expansion of myeloid blasts with suppression of normal haematopoiesis is th...

journal_title：British journal of haematology

authors： Loh ML

更新日期：2011-03-01 00:00:00

abstract：:Excessive bleeding represents a major complication of surgical interventions and its control is especially relevant in patients with Congenital Bleeding Disorders (CBD). In factor VII (FVII) deficiency, scanty data on surgery is available to guide treatment strategies. The STER (Seven Treatment Evaluation Registry) is...

journal_title：British journal of haematology

authors： Mariani G,Dolce A,Batorova A,Auerswald G,Schved JF,Siragusa S,Napolitano M,Knudsen JB,Ingerslev J,STER and the International Factor VII Deficiency Study Groups.

更新日期：2011-02-01 00:00:00

abstract：:Reducing infectious morbidity is an important goal to improve childhood acute lymphoblastic leukaemia (ALL) survival. To explore the impact of chemotherapy reduction on infectious morbidity, we compared outpatient and inpatient infectious morbidity of reduced versus intensive (conventional) chemotherapy. One hundred a...

journal_title：British journal of haematology

authors： van Tilburg CM,Sanders EA,Nibbelke EE,Pieters R,Revesz T,Westers P,Wolfs TF,Bierings MB

更新日期：2011-02-01 00:00:00

abstract：:Hodgkin's lymphoma (HL) can be cured in most of the patients, but in case of refractory disease or relapse after autologous stem cell transplantation (SCT) the prognosis becomes very poor. In these patients a consensus about the standard approach has not been achieved so far and only allogeneic SCT has shown a long-te...

journal_title：British journal of haematology

authors： Corradini P,Sarina B,Farina L

更新日期：2011-02-01 00:00:00

abstract：:The impact of age upon therapeutic response to unfractionated heparin (UFH) in children is proposed to reflect quantitative and potentially qualitative differences in coagulation proteins across childhood. This study explores the UFH-dependent tissue factor pathway inhibitor (TFPI) release in children compared to prev...

journal_title：British journal of haematology

authors： Newall F,Johnston L,Summerhayes R,Lane G,Cranswick N,Monagle P,Ignjatovic V

更新日期：2010-11-01 00:00:00

abstract：:The combination of bortezomib and rituximab was evaluated in patients with mantle cell lymphoma (MCL), follicular lymphoma (FL) and Waldenström macroglobulinaemia (WM), in a Phase I and later, a randomized Phase II study. In the randomized study, 42 patients with recurrent/refractory disease received either: bortezomi...

journal_title：British journal of haematology

authors： Agathocleous A,Rohatiner A,Rule S,Hunter H,Kerr JP,Neeson SM,Matthews J,Strauss S,Montoto S,Johnson P,Radford J,Lister A

更新日期：2010-11-01 00:00:00

abstract：:In acute promyelocytic leukaemia (APL), differentiation therapy can be complicated by the development of a differentiation syndrome (DS). Pulmonary infiltration of differentiating leukaemic cells is a key event in the development of DS. Several mediators have been identified that may promote migration and extravasatio...

journal_title：British journal of haematology

authors： Luesink M,Jansen JH

更新日期：2010-11-01 00:00:00

abstract：:Genetic variations in DNA repair genes are thought to play an important role in the pathogenesis and development of non-Hodgkin lymphoma (NHL). To further explore this hypothesis, we genotyped 319 tag single nucleotide polymorphisms (SNPs) in 27 DNA repair gene regions in 1946 cases and 1808 controls pooled from three...

journal_title：British journal of haematology

authors： Shen M,Menashe I,Morton LM,Zhang Y,Armstrong B,Wang SS,Lan Q,Hartge P,Purdue MP,Cerhan JR,Grulich A,Cozen W,Yeager M,Holford TR,Vajdic CM,Davis S,Leaderer B,Kricker A,Severson RK,Zahm SH,Chatterjee N,Rothman N,

更新日期：2010-11-01 00:00:00