abstract：:Activating mutations of NOTCH1 are a common occurrence in T-cell acute lymphoblastic leukaemia (T-ALL), but its impact on T-ALL treatment is still controversial. In this study, the incidence, clinical features, and prognosis of 92 Chinese children with T-ALL treated using the Beijing Children's Hospital-2003 and Chine...

journal_title：British journal of haematology

authors： Gao C,Liu SG,Zhang RD,Li WJ,Zhao XX,Cui L,Wu MY,Zheng HY,Li ZG

更新日期：2014-07-01 00:00:00

abstract：:Mantle cell lymphoma (MCL) is an aggressive B cell lymphoma, where survival has been remarkably improved by use of protocols including high dose cytarabine, rituximab and autologous stem cell transplantation, such as the Nordic MCL2/3 protocols. In 2008, a MCL international prognostic index (MIPI) was created to enabl...

journal_title：British journal of haematology

authors： Nordström L,Sernbo S,Eden P,Grønbaek K,Kolstad A,Räty R,Karjalainen ML,Geisler C,Ralfkiaer E,Sundström C,Laurell A,Delabie J,Ehinger M,Jerkeman M,Ek S

更新日期：2014-07-01 00:00:00

abstract：:Although expressed in several haematological lineages and involved in multiple different signalling pathways, Bruton tyrosine kinase (BTK) plays an indispensible role in B cells in signalling from the B cell receptor (BCR) for antigen. Many B cell malignancies remain dependent on constitutive BCR signalling, making BT...

journal_title：British journal of haematology

authors： Hutchinson CV,Dyer MJ

更新日期：2014-07-01 00:00:00

abstract：:To assess sources of variability in platelet function tests in normal subjects, 64 healthy young adults were tested on 2-6 occasions at 2 week intervals using four methods: platelet aggregation (AGG) in platelet-rich plasma (PRP) in the Bio/Data PAP-4 Aggregometer (BD) and Chrono-Log Lumi-Aggregometer (CL); and AGG in...

journal_title：British journal of haematology

authors： Miller CH,Rice AS,Garrett K,Stein SF

更新日期：2014-06-01 00:00:00

abstract：:Transplantation of T- and B-cell depleted allografts from haploidentical family donors was evaluated within a prospective phase II trial in children with acute lymphoblastic leukaemia, acute myeloid leukaemia and advanced myelodysplastic syndrome (n = 46). 20 patients had active disease; 19 patients received a second ...

journal_title：British journal of haematology

authors： Lang P,Teltschik HM,Feuchtinger T,Müller I,Pfeiffer M,Schumm M,Ebinger M,Schwarze CP,Gruhn B,Schrauder A,Albert MH,Greil J,Urban C,Handgretinger R

更新日期：2014-06-01 00:00:00

abstract：:Induction of fetal haemoglobin (HbF) is a promising therapeutic approach for the treatment of β-thalassaemia and sickle cell disease (SCD). Several pharmacological agents, such as hydroxycarbamide (HC) and butyrates, have been shown to induce the γ-globin genes (HBG1, HBG2). However, their therapeutic use is limited d...

journal_title：British journal of haematology

authors： Ronzoni L,Sonzogni L,Fossati G,Modena D,Trombetta E,Porretti L,Cappellini MD

更新日期：2014-06-01 00:00:00

abstract：:The role of autologous stem cell transplantation (ASCT) and the optimal timing of such transplants in patients with follicular lymphoma (FL) remains contentious. We present a single-centre experience documenting the outcomes of 70 FL patients who underwent BEAM (carmustine, cytarabine, etopside, melphalan)-conditioned...

journal_title：British journal of haematology

authors： Kothari J,Peggs KS,Bird A,Thomson KJ,Morris E,Virchis AE,Lambert J,Goldstone AH,Linch DC,Ardeshna KM

更新日期：2014-05-01 00:00:00

abstract：:Classical Hodgkin lymphoma (cHL) is characterized by a paucity of neoplastic Hodgkin/Reed Sternberg (HRS) cells within a complex cellular milieu that is rendered immunologically incapable of reacting against CD30(+) HRS cells due to a plethora of immune escape mechanisms initiated by the neoplastic cells. Accounting f...

journal_title：British journal of haematology

authors： Venkataraman G,Mirza MK,Eichenauer DA,Diehl V

更新日期：2014-05-01 00:00:00

abstract：:Adult survivors of childhood acute lymphoblastic leukaemia (ALL) have a four-fold excess risk of mortality from cardiovascular disease. This cardiovascular risk has not been fully characterized. ALL survivors [n = 784, median age 31·7 years (18·9-59·1)] in the St. Jude Lifetime Cohort Study underwent evaluation for ca...

journal_title：British journal of haematology

authors： Nottage KA,Ness KK,Li C,Srivastava D,Robison LL,Hudson MM

更新日期：2014-05-01 00:00:00

abstract：:Primary familial and congenital polycythaemia (PFCP) is a disease characterized by increased red blood cell mass, and can be associated with mutations in the intracellular region of the erythropoietin (EPO) receptor (EPOR). Here we explore the mechanisms by which EPOR mutations induce PFCP, using an experimental syste...

journal_title：British journal of haematology

authors： Gross M,Ben-Califa N,McMullin MF,Percy MJ,Bento C,Cario H,Minkov M,Neumann D

更新日期：2014-05-01 00:00:00

abstract：:Diffuse large B-cell lymphoma (DLBCL) is a heterogeneous disease and "double-hit" DLBCL, with both MYC and BCL2 translocations has a poor prognosis. In this study, we investigated whether MYC and BCL2 protein expression in tissue would predict survival in DLBCL. The study included 106 cases of de novo DLBCL treated wi...

journal_title：British journal of haematology

authors： Perry AM,Alvarado-Bernal Y,Laurini JA,Smith LM,Slack GW,Tan KL,Sehn LH,Fu K,Aoun P,Greiner TC,Chan WC,Bierman PJ,Bociek RG,Armitage JO,Vose JM,Gascoyne RD,Weisenburger DD

更新日期：2014-05-01 00:00:00

abstract：:The role of apoptotic pathways in the development and function of the megakaryocyte lineage has generated renewed interest in recent years. This has been driven by the advent of BH3 mimetic drugs that target BCL2 family proteins to induce apoptosis in tumour cells: agents such as ABT-263 (navitoclax, which targets BCL...

journal_title：British journal of haematology

authors： Kile BT

更新日期：2014-04-01 00:00:00

abstract：:Our knowledge of the cellular and molecular mechanisms of platelet production has greatly expanded in recent years due to the opportunity to culture in vitro megakaryocytes and to create transgenic animals with specific genetic defects that interfere with platelet biogenesis. However, in vitro models do not reproduce ...

journal_title：British journal of haematology

authors： Pecci A,Balduini CL

更新日期：2014-04-01 00:00:00

abstract：:The significance of paroxysmal nocturnal haemoglobinuria (PNH(pos) ) cells and leucocyte subset telomere lengths in paediatric aplastic anaemia (AA) is unknown. Among 22 children receiving immunosuppressive therapy (IST) for AA, 73% (16/22) were PNH(pos) , of whom 94% achieved at least a partial response (PR) to IST; ...

journal_title：British journal of haematology

authors： Tutelman PR,Aubert G,Milner RA,Dalal BI,Schultz KR,Deyell RJ

更新日期：2014-03-01 00:00:00

abstract：:ATOH8 has previously been shown to be an iron-regulated transcription factor, however its role in iron metabolism is not known. ATOH8 expression in HEK293 cells resulted in increased endogenous HAMP mRNA levels as well as HAMP promoter activity. Mutation of the E-box or SMAD response elements within the HAMP promoter ...

journal_title：British journal of haematology

authors： Patel N,Varghese J,Masaratana P,Latunde-Dada GO,Jacob M,Simpson RJ,McKie AT

更新日期：2014-02-01 00:00:00

abstract：:Novel therapies with increased efficacy and decreased toxicity are desperately needed for the treatment of acute myeloid leukaemia (AML). The anti CD33 immunoconjugate, gemtuzumab ozogamicin (GO), was withdrawn with concerns over induction mortality and lack of efficacy. However a number of recent trials suggest that,...

journal_title：British journal of haematology

authors： Gasiorowski RE,Clark GJ,Bradstock K,Hart DN

更新日期：2014-02-01 00:00:00

abstract：:International guidance has recently recommended serial proximal compression ultrasound (CUS) as first line imaging for suspected deep vein thrombosis (DVT). Single whole-leg CUS is a routine alternative diagnostic strategy that can reduce repeated attendances and identify alternative pathology. We conducted a prospect...

journal_title：British journal of haematology

authors： Horner D,Hogg K,Body R,Jones S,Nash MJ,Mackway-Jones K

更新日期：2014-02-01 00:00:00

abstract：:Sickle cell disease (SCD) in Saudi patients from the Eastern Province is associated with the Arab-Indian (AI) HBB (β-globin gene) haplotype. The phenotype of AI SCD in children was described as benign and was attributed to their high fetal haemoglobin (HbF). We conducted a hospital-based study to assess the pattern of...

journal_title：British journal of haematology

authors： Alsultan A,Alabdulaali MK,Griffin PJ,Alsuliman AM,Ghabbour HA,Sebastiani P,Albuali WH,Al-Ali AK,Chui DH,Steinberg MH

更新日期：2014-02-01 00:00:00

abstract：:Very few studies have investigated the effects of exercise on the biological parameters involved in vaso-occlusive events in sickle cell anaemia (SCA). The aim of this study was to test how a mild-moderate endurance exercise modulates oxidative stress, nitric oxide bioavailability and endothelial activation in SCA pat...

journal_title：British journal of haematology

authors： Faes C,Balayssac-Siransy E,Connes P,Hivert L,Danho C,Bogui P,Martin C,Pialoux V

更新日期：2014-01-01 00:00:00

abstract：:Acute promyelocytic leukaemia (APL) is a rare subtype of acute myeloid leukaemia. The outcome of paediatric APL has improved substantially over the past 20 years; cure rates above 80% are expected when all-trans retinoic acid (ATRA) is given with anthracycline-based regimens. The presenting features of paediatric APL ...

journal_title：British journal of haematology

authors： Abla O,Ribeiro RC

更新日期：2014-01-01 00:00:00

abstract：:UK National External Quality Assessment Service surveys indicate continuing problems in malaria diagnosis: inaccurate calculation of parasitaemia or failure to estimate it altogether, difficulty distinguishing Plasmodium vivax from P. ovale, reporting malaria parasites when none were present and misidentification of P...

journal_title：British journal of haematology

authors： Bailey JW,Williams J,Bain BJ,Parker-Williams J,Chiodini PL,General Haematology Task Force of the British Committee for Standards in Haematology.

更新日期：2013-12-01 00:00:00

abstract：:In patients with sickle cell anaemia (SCA), concomitant glucose-6-phosphate dehydrogenase (G6PD) deficiency is usually described as having no effect and only occasionally as increasing severity. We analysed sequential clinical and biological data for the first 42 months of life in SCA patients diagnosed by neonatal sc...

journal_title：British journal of haematology

authors： Benkerrou M,Alberti C,Couque N,Haouari Z,Ba A,Missud F,Boizeau P,Holvoet L,Ithier G,Elion J,Baruchel A,Ducrocq R

更新日期：2013-12-01 00:00:00

abstract：:Detection of a 17p13.1 deletion (loss of TP53) or 11q22.3 deletion (loss of ATM), by fluorescence in situ hybridization (FISH), in chronic lymphocytic leukaemia (CLL) patients is associated with a poorer prognosis. Because TP53 and ATM are integral to the TP53 pathway, we hypothesized that 17p13.1- (17p-) and 11q22.3-...

journal_title：British journal of haematology

authors： Greipp PT,Smoley SA,Viswanatha DS,Frederick LS,Rabe KG,Sharma RG,Slager SL,Van Dyke DL,Shanafelt TD,Tschumper RC,Zent CS

更新日期：2013-11-01 00:00:00

abstract：:Laboratory (LTLS) and clinical (CTLS) tumour lysis syndrome (TLS) are frequent complications in newly diagnosed children with advanced mature B cell non-Hodgkin lymphoma (B-NHL). Rasburicase, compared to allopurinol, results in more rapid reduction of uric acid in paediatric patients at risk for TLS. However, the safe...

journal_title：British journal of haematology

authors： Galardy PJ,Hochberg J,Perkins SL,Harrison L,Goldman S,Cairo MS

更新日期：2013-11-01 00:00:00

abstract：:Ethnic variations in red blood cell (RBC) antigens can be a source of alloimmunization, especially in migrant populations. To improve transfusion safety in continental Africa and countries with African migrants, we performed RBC genotyping to determine allele frequencies coding for high- and low-prevalence antigens. A...

journal_title：British journal of haematology

authors： Silvy M,Beley S,Granier T,Ba A,Chiaroni J,Bailly P

更新日期：2013-11-01 00:00:00

abstract：:Evidence regarding the efficacy of gemtuzumab ozogamicin (GO) addition to standard induction chemotherapy in newly diagnosed acute myeloid leukaemia (AML) is conflicting. This systematic review aimed to identify and summarize all evidence regarding the benefits and harms of adding GO to conventional chemotherapy for i...

journal_title：British journal of haematology

authors： Kharfan-Dabaja MA,Hamadani M,Reljic T,Pyngolil R,Komrokji RS,Lancet JE,Fernandez HF,Djulbegovic B,Kumar A

更新日期：2013-11-01 00:00:00

abstract：:Over the past decades, a wealth of information has been reported about the pathogenic features of immune thrombocytopenia (ITP). To this day, however, it is unclear whether the immune abnormalities associated with ITP play causative roles in the disease or are secondary epiphenomena brought on by the inflammatory proc...

journal_title：British journal of haematology

authors： McKenzie CG,Guo L,Freedman J,Semple JW

更新日期：2013-10-01 00:00:00

abstract：:Clofarabine was the latest new drug to be approved, in 2004, for relapsed or refractory acute lymphoblastic leukaemia (ALL). To investigate its value in the frontline treatment of ALL we applied clofarabine 5 × 40 mg/m(2) in combination with pegylated asparaginase (PEG-ASP) 1 × 2500 iu/m(2) in high risk ALL patients a...

journal_title：British journal of haematology

authors： Escherich G,Zur Stadt U,Zimmermann M,Horstmann MA,CoALL study group.

更新日期：2013-10-01 00:00:00

abstract：:Amiodarone has been implicated as a cause of thrombocytopenia but the responsible mechanism is unknown. We performed studies in three patients to characterize the pathogenesis of this complication. No amiodarone-dependent, platelet-reactive antibodies were identified using conventional serological techniques. However,...

journal_title：British journal of haematology

authors： Sahud MA,Caulfield M,Clarke N,Koch R,Bougie D,Aster R

更新日期：2013-10-01 00:00:00

abstract：:Losses in 13q as a sole abnormality confer a good prognosis in chronic lymphocytic leukaemia (CLL). Nevertheless, its heterogeneity has been demonstrated and the clinical significance of biallelic 13q deletions remains controversial. We compared the clinico-biological characteristics of a series of 627 patients harbou...

journal_title：British journal of haematology

authors： Puiggros A,Delgado J,Rodriguez-Vicente A,Collado R,Aventín A,Luño E,Grau J,Hernandez JÁ,Marugán I,Ardanaz M,González T,Valiente A,Osma M,Calasanz MJ,Sanzo C,Carrió A,Ortega M,Santacruz R,Abrisqueta P,Abella E,Bosc

更新日期：2013-10-01 00:00:00

abstract：:Polo-like kinases (Plks) play an important role in cell cycle checkpoint controls and are over-expressed in acute myeloid leukaemia (AML). BI 2536, a novel Plk inhibitor, induces mitotic arrest and apoptosis. In this phase I/II trial of BI 2536 in 68 elderly patients with relapsed/refractory AML, three schedules were ...

journal_title：British journal of haematology

authors： Müller-Tidow C,Bug G,Lübbert M,Krämer A,Krauter J,Valent P,Nachbaur D,Berdel WE,Ottmann OG,Fritsch H,Munzert G,Garin-Chesa P,Fleischer F,Taube T,Döhner H

更新日期：2013-10-01 00:00:00

abstract：:Haematopoietic cell transplantation (HCT) survivors are at increased risk for developing congestive heart failure (CHF), primarily due to pre-HCT exposure to anthracyclines. We examined the association between the development of CHF after HCT and polymorphisms in 16 candidate genes involved in anthracycline metabolism...

journal_title：British journal of haematology

authors： Armenian SH,Ding Y,Mills G,Sun C,Venkataraman K,Wong FL,Neuhausen SL,Senitzer D,Wang S,Forman SJ,Bhatia S

更新日期：2013-10-01 00:00:00

abstract：:Splanchnic vein thrombosis (SVT) is one of the most important complications of myeloproliferative neoplasms (MPN). Although MPN are common causes of SVT, the pathophysiological mechanisms underlying this predisposition, their epidemiology and natural history are not fully understood. Studies have concentrated on the g...

journal_title：British journal of haematology

authors： Sekhar M,McVinnie K,Burroughs AK

更新日期：2013-09-01 00:00:00

abstract：:There are no accepted methods to predict the development of platelet transfusion refractoriness (PTR) due to human leucocyte antigen (HLA)-alloimmunization. Hence, matched platelets are usually given only to patients demonstrating PTR, necessarily resulting in some ineffective random donor platelets (RDPLT) transfusio...

journal_title：British journal of haematology

authors： Beligaswatte A,Tsiopelas E,Humphreys I,Bennett G,Robinson K,Davis K,Bardy P

更新日期：2013-08-01 00:00:00

abstract：:Chronic lymphocytic leukaemia/small lymphocytic lymphoma (CLL) is one of the more common forms of B cell malignancy. Although the condition has a variable clinical course, the trend is towards eventual relapse and the disease is considered incurable. Whilst the majority of the circulating CD5-positive neoplastic B cel...

journal_title：British journal of haematology

authors： Ramsay AD,Rodriguez-Justo M

更新日期：2013-07-01 00:00:00

abstract：:Children with Down syndrome (DS) have a greater risk for developing both acute lymphoblastic leukaemia (ALL) and significant adverse effects of chemotherapy. We investigated their outcome with, and tolerance of, treatment protocols for relapsed ALL optimized in the paediatric population without DS. Probability of surv...

journal_title：British journal of haematology

authors： Meyr F,Escherich G,Mann G,Klingebiel T,Kulozik A,Rossig C,Schrappe M,Henze G,von Stackelberg A,Hitzler J

更新日期：2013-07-01 00:00:00

abstract：:Peripheral blood haematopoietic progenitor cell mobilization has become a standard procedure prior to autologous stem cell transplantation. Biosimilar granulocyte colony-stimulating factors (GCSF) have recently been awarded European Union (EU) licences for stem cell mobilization but data for their use in this context ...

journal_title：British journal of haematology

authors： Publicover A,Richardson DS,Davies A,Hill KS,Hurlock C,Hutchins D,Jenner MW,Johnson PW,Lamb J,Launders H,McKeag N,Newman J,Orchard KH

更新日期：2013-07-01 00:00:00

abstract：:Givinostat, a histone-deacetylase inhibitor (HDACi), inhibits proliferation of cells bearing the JAK2 V617F mutation and has shown significant activity with good tolerability in patients with chronic myeloproliferative neoplasms (MPN). In this multicentre, open-label, phase II study, 44 patients with polycythaemia ver...

journal_title：British journal of haematology

authors： Finazzi G,Vannucchi AM,Martinelli V,Ruggeri M,Nobile F,Specchia G,Pogliani EM,Olimpieri OM,Fioritoni G,Musolino C,Cilloni D,Sivera P,Barosi G,Finazzi MC,Di Tollo S,Demuth T,Barbui T,Rambaldi A

更新日期：2013-06-01 00:00:00

abstract：:Interleukin 17A (IL17A), a cytokine involved in allergy, inflammation and osteoclastogenesis, was investigated in multiple myeloma (MM) to assess its role in the osteoclast (OC)-like activity of marrow immature dendritic cells (iDCs). Comparing nine MM patients with control subjects affected by monoclonal gammopathy o...

journal_title：British journal of haematology

authors： Tucci M,Stucci S,Savonarola A,Ciavarella S,Cafforio P,Dammacco F,Silvestris F

更新日期：2013-06-01 00:00:00

abstract：:To determine the long-term efficacy of FLT3 inhibitors (FLT3i) in the salvage setting for relapsed and refractory (rel/ref) acute myeloid leukemia (AML) with FLT3 internal tandem duplication (AML FLT3-ITD), we conducted a retrospective study of 120 patients with rel/ref AML FLT3-ITD who received salvage therapy with e...

journal_title：British journal of haematology

authors： Takahashi K,Kantarjian H,Pemmaraju N,Andreeff M,Borthakur G,Faderl S,Garcia-Manero G,Pierce S,Luthra R,Cardenas-Turanzas M,Estrov Z,Ravandi F,Cortes J

更新日期：2013-06-01 00:00:00