abstract：:Clinical management of chronic lymphocytic leukaemia (CLL) in patients aged ≥80 years is based on limited evidence due to the lack of published information. Therefore, we analysed CLL patients aged ≥80 years using data from seven phase III clinical trials of the German CLL Study Group. Among 3552 participants, 152 wer...

journal_title：British journal of haematology

authors： Al-Sawaf O,Bahlo J,Robrecht S,Fischer K,Herling CD,Hoechstetter M,Fink AM,von Tresckow J,Langerbeins P,Cramer P,Stilgenbauer S,Wendtner CM,Eichhorst B,Hallek M,Goede V

更新日期：2018-12-01 00:00:00

abstract：:Long term outcomes and mutations in patients with mantle cell lymphoma (MCL) who discontinued ibrutinib have not been described. Using deep targeted next generation sequencing, we performed somatic mutation profiling from 15 MCL patients (including 5 patients with paired samples; before and after progression on ibruti...

journal_title：British journal of haematology

authors： Jain P,Kanagal-Shamanna R,Zhang S,Ahmed M,Ghorab A,Zhang L,Ok CY,Li S,Hagemeister F,Zeng D,Gong T,Chen W,Badillo M,Nomie K,Fayad L,Medeiros LJ,Neelapu S,Fowler N,Romaguera J,Champlin R,Wang L,Wang ML

更新日期：2018-11-01 00:00:00

abstract：:Over the past four decades, remarkable progress has been made in the treatment and prognosis of multiple myeloma (MM), although it remains an incurable disease. Chemotherapy resistance is a major hurdle for treatment efficacy. Drug resistance can be innate and so driven by genes involved in the drug metabolism pathway...

journal_title：British journal of haematology

authors： Macauda A,Castelli E,Buda G,Pelosini M,Butrym A,Watek M,Kruszewski M,Vangsted AJ,Rymko M,Jamroziak K,Abildgaard N,Haastrup EK,Mazur G,Ríos R,Jurczyszyn A,Zawirska D,Dudziński M,Raźny M,Dutka M,Tomczak W,Suska A,

更新日期：2018-11-01 00:00:00

abstract：:The mechanisms involved in regulating von Willebrand factor (VWF) clearance remain poorly understood. However recent studies have shown that macrophages play a critical role in regulating the half-life of VWF, and have identified specific lectin (including asialoglycoprotein, macrophage galactose-type lectin, Sigec-5 ...

journal_title：British journal of haematology

authors： O'Sullivan JM,Ward S,Lavin M,O'Donnell JS

更新日期：2018-10-01 00:00:00

abstract：:Increasing use of direct oral anticoagulants (DOACs) has made management of non-valvular atrial fibrillation and venous thromboembolism easier in most patients. But the presence of co-existing renal impairment could render the use of DOACs problematic because all of these drugs have varying degrees of renal excretion....

journal_title：British journal of haematology

authors： Parker K,Thachil J

更新日期：2018-10-01 00:00:00

abstract：:Notwithstanding the improvement in treatment results for paediatric T cell acute lymphoblastic leukaemia (T-ALL) it remains important to understand if genetic aberrations influence therapy response. PTEN tumour suppressor gene inactivation is a frequent event in T-ALL but its effect on patient therapy response is deba...

journal_title：British journal of haematology

authors： Paganin M,Grillo MF,Silvestri D,Scapinello G,Buldini B,Cazzaniga G,Biondi A,Valsecchi MG,Conter V,Te Kronnie G,Basso G

更新日期：2018-09-01 00:00:00

abstract：:In the Medical Research Council (MRC) Myeloma IX trial (ISRCTN684564111) patients were randomised to sodium clodronate or zoledronic acid and induction treatment: cyclophosphamide, vincristine, doxorubicin and dexamethasone (CVAD) or cyclophosphamide, thalidomide and dexamethasone (CTD) followed by autologous stem cel...

journal_title：British journal of haematology

authors： Royle KL,Gregory WM,Cairns DA,Bell SE,Cook G,Owen RG,Drayson MT,Davies FE,Jackson GH,Morgan GJ,Child JA

更新日期：2018-09-01 00:00:00

abstract：:Isolated trisomy 8 (+8) is a frequent cytogenetic abnormality in the myelodysplastic syndromes (MDS), but its characteristics are poorly reported. We performed a retrospective study of 138 MDS patients with isolated +8, classified or reclassified as MDS (excluding MDS/myeloproliferative neoplasm). Myeloproliferative (...

journal_title：British journal of haematology

authors： Drevon L,Marceau A,Maarek O,Cuccuini W,Clappier E,Eclache V,Cluzeau T,Richez V,Berkaoui I,Dimicoli-Salazar S,Bidet A,Vial JP,Park S,Vieira Dos Santos C,Kaphan E,Berthon C,Stamatoullas A,Delhommeau F,Abermil N,Braun

更新日期：2018-09-01 00:00:00

abstract：:Diagnosing central nervous system (CNS) lymphoma remains a challenge. Most patients have to undergo brain biopsy to obtain tissue for diagnosis, with associated risks of serious complications. Diagnostic markers in blood or cerebrospinal fluid (CSF) could facilitate early diagnosis with low complication rates. We perf...

journal_title：British journal of haematology

authors： van Westrhenen A,Smidt LCA,Seute T,Nierkens S,Stork ACJ,Minnema MC,Snijders TJ

更新日期：2018-08-01 00:00:00

abstract：:Establishing a precise diagnosis is essential in inborn haematological cytopenias to enable appropriate treatment decisions and avoid secondary organ damage. However, both diversity and phenotypic overlap of distinct disease entities may make the identification of underlying genetic aetiologies by classical Sanger seq...

journal_title：British journal of haematology

authors： Kager L,Jimenez Heredia R,Hirschmugl T,Dmytrus J,Krolo A,Müller H,Bock C,Zeitlhofer P,Dworzak M,Mann G,Holter W,Haas O,Boztug K

更新日期：2018-07-01 00:00:00

abstract：:The number of novel therapies for the treatment of myeloma is rapidly increasing, as are the clinical trials evaluating them in combination with other novel and established therapies. Proteasome inhibitors, immunomodulatory agents and monoclonal antibodies are the most well known and studied classes of novel agents ta...

journal_title：British journal of haematology

authors： Wallington-Beddoe CT,Sobieraj-Teague M,Kuss BJ,Pitson SM

更新日期：2018-07-01 00:00:00

abstract：:In recent years there has been a paradigm shift in our understanding of the epidemiology and clinical features of hepatitis E virus (HEV) infection. Once classically described as an acute hepatitis associated with waterborne outbreaks in areas of poor sanitation, HEV is now recognised to be endemic in Europe and is pr...

journal_title：British journal of haematology

authors： O'Gorman J,Burke Á,O'Flaherty N

更新日期：2018-06-01 00:00:00

abstract：:Despite the significance of red blood cell (RBC) alloimmunization, the lack of standardized registries in the US has prevented the completion of large studies. Data from 3·5 years of the Recipient Epidemiology and Donor Evaluation Study-III (REDS-III) recipient database, containing information from 12 hospitals, were ...

journal_title：British journal of haematology

authors： Karafin MS,Westlake M,Hauser RG,Tormey CA,Norris PJ,Roubinian NH,Wu Y,Triulzi DJ,Kleinman S,Hendrickson JE,NHLBI Recipient Epidemiology and Donor Evaluation Study-III (REDS-III).

更新日期：2018-06-01 00:00:00

abstract：:Recurrent large-scale somatic copy number alterations (SCNAs), and somatic point mutations can be analysed to stratify patients with chronic lymphocytic leukaemia (CLL) into distinct prognostic groups. To investigate the relationship between SCNAs and somatic mutations, we performed whole-exome sequencing and single n...

journal_title：British journal of haematology

authors： Zhou W,Goldin L,Wang M,McMaster ML,Jones K,Burdett L,Chanock SJ,Yeager M,Dean M,Caporaso NE

更新日期：2018-06-01 00:00:00

abstract：:Serum ferritin level is one of the most commonly requested investigations in both primary and secondary care. Whilst low serum ferritin levels invariably indicate reduced iron stores, raised serum ferritin levels can be due to multiple different aetiologies, including iron overload, inflammation, liver or renal diseas...

journal_title：British journal of haematology

authors： Cullis JO,Fitzsimons EJ,Griffiths WJ,Tsochatzis E,Thomas DW,British Society for Haematology.

更新日期：2018-05-01 00:00:00

abstract：:T cells modified with anti-CD19 chimeric antigen receptor (CAR) containing either CD28 or 4-1BB (also termed TNFRSF9, CD137) costimulatory signalling have shown great potential in the treatment of acute lymphoblastic leukaemia (ALL). However, the difference between CD28 and 4-1BB costimulatory signalling in CAR-T trea...

journal_title：British journal of haematology

authors： Li S,Zhang J,Wang M,Fu G,Li Y,Pei L,Xiong Z,Qin D,Zhang R,Tian X,Wei Z,Chen R,Chen X,Wan J,Chen J,Wei X,Xu Y,Zhang P,Wang P,Peng X,Yang S,Shen J,Yang Z,Chen J,Qian C

更新日期：2018-05-01 00:00:00

abstract：:This trial explored the efficacy of re-induction chemotherapy including bortezomib in paediatric relapsed/refractory acute lymphoblastic leukaemia. Patients were randomized 1:1 to bortezomib (1.3 mg/m2 /dose) administered early or late to a dexamethasone and vincristine backbone. Both groups did not differ regarding p...

journal_title：British journal of haematology

authors： Kaspers GJL,Niewerth D,Wilhelm BAJ,Scholte-van Houtem P,Lopez-Yurda M,Berkhof J,Cloos J,de Haas V,Mathôt RA,Attarbaschi A,Baruchel A,de Bont ES,Fagioli F,Rössig C,Klingebiel T,De Moerloose B,Nelken B,Palumbo G,Reinhar

更新日期：2018-05-01 00:00:00

abstract：:Relapse remains the leading cause of treatment failure in children with acute lymphoblastic leukaemia (ALL) undergoing allogeneic haematopoietic stem cell transplantation (HSCT). We retrospectively investigated the prognostic role of minimal residual disease (MRD) before and after HSCT in 119 children transplanted in ...

journal_title：British journal of haematology

authors： Lovisa F,Zecca M,Rossi B,Campeggio M,Magrin E,Giarin E,Buldini B,Songia S,Cazzaniga G,Mina T,Acquafredda G,Quarello P,Locatelli F,Fagioli F,Basso G

更新日期：2018-03-01 00:00:00

abstract：:MYD88 mutations are present in 95% of Waldenstrom Macroglobulinaemia (WM) patients, and support diagnostic discrimination from other IgM-secreting B-cell malignancies. Diagnostic discrimination can be difficult among suspected wild-type MYD88 (MYD88WT ) WM cases. We systematically reviewed the clinical, pathological a...

journal_title：British journal of haematology

authors： Treon SP,Gustine J,Xu L,Manning RJ,Tsakmaklis N,Demos M,Meid K,Guerrera ML,Munshi M,Chan G,Chen J,Kofides A,Patterson CJ,Yang G,Liu X,Severns P,Dubeau T,Hunter ZR,Castillo JJ

更新日期：2018-02-01 00:00:00

abstract：:The outcomes of adult patients transplanted for Fanconi anaemia (FA) have not been well described. We retrospectively analysed 199 adult patients with FA transplanted between 1991 and 2014. Patients were a median of 16 years of age when diagnosed with FA, and underwent transplantation at a median age of 23 years. Time...

journal_title：British journal of haematology

authors： Bierings M,Bonfim C,Peffault De Latour R,Aljurf M,Mehta PA,Knol C,Boulad F,Tbakhi A,Esquirol A,McQuaker G,Sucak GA,Othman TB,Halkes CJM,Carpenter B,Niederwieser D,Zecca M,Kröger N,Michallet M,Risitano AM,Ehninger G

更新日期：2018-01-01 00:00:00

abstract：:Marizomib (MRZ) is an irreversible, pan-subunit proteasome inhibitor (PI) in clinical development for relapsed/refractory multiple myeloma (RRMM) and glioma. This study analysed MRZ, pomalidomide (POM) and low-dose dexamethasone (Lo-DEX) [PMD] in RRMM to evaluate safety and determine the maximum tolerated dose (MTD) a...

journal_title：British journal of haematology

authors： Spencer A,Harrison S,Zonder J,Badros A,Laubach J,Bergin K,Khot A,Zimmerman T,Chauhan D,Levin N,MacLaren A,Reich SD,Trikha M,Richardson P

更新日期：2018-01-01 00:00:00

abstract：:The in vitro haemostatic functions of fresh whole blood (FWB) are well preserved after cold storage. This study aimed to determine whether platelets derived from FWB and stored whole blood (SWB) contribute to clot formation in tissue injury after transfusion into coagulopathic rats with polytrauma/haemorrhage (T/H). T...

journal_title：British journal of haematology

authors： Wu X,Darlington DN,Montgomery RK,Liu B,Keesee JD,Scherer MR,Benov A,Chen J,Cap AP

更新日期：2017-12-01 00:00:00

abstract：:The laboratory diagnosis of heparin-induced thrombocytopenia (HIT) is based on an enzyme immunoassay combined with a functional test, and serotonin release assay (SRA) is the gold standard for detecting activating HIT antibodies. However, a recent atypical history of HIT prompted us to evaluate whether addition of pla...

journal_title：British journal of haematology

authors： Vayne C,Guery EA,Kizlik-Masson C,Rollin J,Bauters A,Gruel Y,Pouplard C

更新日期：2017-12-01 00:00:00

abstract：:Thrombocytopenia absent radii (TAR) syndrome is clearly defined by the combination of radial aplasia and reduced platelet counts. The genetics of TAR syndrome has recently been resolved and comprises a microdeletion on Chromosome 1 including the RBM8A gene and a single nucleotide polymorphism (SNP) either at the 5' un...

journal_title：British journal of haematology

authors： Manukjan G,Bösing H,Schmugge M,Strauß G,Schulze H

更新日期：2017-11-01 00:00:00

abstract：:Comparative data on immunochemotherapy regimens for Waldenström macroglobulinaemia/lymphoplasmacytic lymphoma (WM/LPL) are lacking. We analysed overall survival (OS), risk of hospitalizations, transfusions and plasmapheresis in a population-based cohort of patients ≥65 years old initiating WM/LPL therapy in 1999-2013....

journal_title：British journal of haematology

authors： Olszewski AJ,Chen C,Gutman R,Treon SP,Castillo JJ

更新日期：2017-10-01 00:00:00

abstract：:The availability of novel therapies for the treatment of multiple myeloma has had a dramatic impact on the depth of response that can be expected on initial treatment. Despite these advances, disease relapse remains inevitable in most patients and brings with it a different set of priorities for therapy. The most rece...

journal_title：British journal of haematology

authors： Moreau P,de Wit E

更新日期：2017-10-01 00:00:00

abstract：:Panobinostat in combination with bortezomib and dexamethasone demonstrated a significant and clinically meaningful progression-free survival benefit compared with placebo, bortezomib and dexamethasone in the phase 3 PANORAMA 1 (Panobinostat Oral in Multiple Myeloma 1) trial. Despite this benefit, patients in the panob...

journal_title：British journal of haematology

authors： San-Miguel JF,Hungria VTM,Yoon SS,Beksac M,Dimopoulos MA,Elghandour A,Jedrzejczak WW,Guenther A,Na Nakorn T,Siritanaratkul N,Schlossman RL,Hou J,Moreau P,Lonial S,Lee JH,Einsele H,Salwender H,Sopala M,Redhu S,Paul S

更新日期：2017-10-01 00:00:00

abstract：:High frequency of periodic limb movements in sleep (PLMS) has been described among children with sickle cell disease (SCD), but there is little information about PLMS among adults with SCD. We aim to determine the frequency of PLMS among adults with SCD and to identify possible associations with iron status and haemol...

journal_title：British journal of haematology

authors： Cabañas-Pedro AC,Roizenblatt S,de Souza AAL,Tufik S,Figueiredo MS

更新日期：2017-10-01 00:00:00

abstract：:The significance of serum lactate dehydrogenase (LDH) in light chain (AL) amyloidosis has not been previously explored. We studied 1019 newly diagnosed patients and correlated the elevation of LDH above the upper limit of normal (ULN) with disease characteristics and outcome. Four hundred and nine patients had an LDH ...

journal_title：British journal of haematology

authors： Muchtar E,Dispenzieri A,Lacy MQ,Buadi FK,Kapoor P,Hayman SR,Gonsalves W,Warsame R,Kourelis TV,Chakraborty R,Russell S,Lust JA,Lin Y,Go RS,Zeldenrust S,Dingli D,Leung N,Rajkumar SV,Kyle RA,Kumar SK,Gertz MA

更新日期：2017-09-01 00:00:00

abstract：:Massive genomic analyses have underscored the diversity of chronic lymphocytic leukaemia (CLL) between patients. Genetic heterogeneity of tumour clones within a patient may fuel tumour evolution. Several recurrently deregulated intra-cellular pathways are candidates for targeted therapies that are very promising and a...

journal_title：British journal of haematology

authors： Ghamlouch H,Nguyen-Khac F,Bernard OA

更新日期：2017-09-01 00:00:00

abstract：:Anaplastic large cell lymphomas (ALCLs) are rare CD30+ peripheral T-cell lymphomas (PTCLs) classified according to the expression of the anaplastic lymphoma kinase (ALK+) protein or not (ALK-). We have analysed the outcome and risk factors for survival in a population-based bi-national cohort of patients with systemic...

journal_title：British journal of haematology

authors： Cederleuf H,Bjerregård Pedersen M,Jerkeman M,Relander T,d'Amore F,Ellin F

更新日期：2017-09-01 00:00:00

abstract：:Lenalidomide (LEN) acts directly on multiple myeloma (MM) cells by inducing cereblon-mediated degradation of interferon regulatory factor 4, Ikaros (IKZF)1 and IKZF3, transcription factors that are essential for MM cell survival. The mucin 1 (MUC1) C-terminal transmembrane subunit (MUC1-C) oncoprotein is aberrantly ex...

journal_title：British journal of haematology

authors： Yin L,Tagde A,Gali R,Tai YT,Hideshima T,Anderson K,Avigan D,Kufe D

更新日期：2017-09-01 00:00:00

abstract：:There is limited information concerning the impact of physical activity and obesity on non-Hodgkin lymphoma (NHL) prognosis. We examined the associations between pre-diagnosis physical activity and body mass index (BMI) with survival in 238 diffuse large B-cell (DLBCL) and 175 follicular lymphoma cases, with follow-up...

journal_title：British journal of haematology

authors： Boyle T,Connors JM,Gascoyne RD,Berry BR,Sehn LH,Bashash M,Spinelli JJ

更新日期：2017-08-01 00:00:00

abstract：:Multiple Myeloma (MM) is a haematological malignancy characterised by the clonal expansion of plasma cells (PCs) within the bone marrow. Despite advances in therapy, MM remains a largely incurable disease with a median survival of 6 years. In almost all cases, the development of MM is preceded by the benign PC conditi...

journal_title：British journal of haematology

authors： Dutta AK,Hewett DR,Fink JL,Grady JP,Zannettino ACW

更新日期：2017-07-01 00:00:00

abstract：:The variable clinical outcomes of Multiple Myeloma (MM) patients are incompletely defined by current prognostication tools. We examined the clinical utility of high-resolution telomere length analysis as a prognostic marker in MM. Cohort stratification, using a previously determined length threshold for telomere dysfu...

journal_title：British journal of haematology

authors： Hyatt S,Jones RE,Heppel NH,Grimstead JW,Fegan C,Jackson GH,Hills R,Allan JM,Pratt G,Pepper C,Baird DM

更新日期：2017-07-01 00:00:00

abstract：:This study compared the diagnostic value of Whole-Body Ultra Low-Dose computed tomography (WBULDCT) with that of Spinal Magnetic Resonance Imaging (SMRI) in identification of spinal bone marrow involvement in patients with Multiple Myeloma (MM). Thirty-five patients with histologically proven MM underwent WBULDCT and ...

journal_title：British journal of haematology

authors： Ippolito D,Talei Franzesi C,Spiga S,Besostri V,Pezzati S,Rossini F,Sironi S

更新日期：2017-05-01 00:00:00

abstract：:A primary analysis of the ASPIRE study found that the addition of carfilzomib to lenalidomide and dexamethasone (carfilzomib group) significantly improved progression-free survival (PFS) compared with lenalidomide and dexamethasone alone (control group) in patients with relapsed multiple myeloma (RMM). This post hoc a...

journal_title：British journal of haematology

authors： Dimopoulos MA,Stewart AK,Masszi T,Špička I,Oriol A,Hájek R,Rosiñol L,Siegel D,Mihaylov GG,Goranova-Marinova V,Rajnics P,Suvorov A,Niesvizky R,Jakubowiak A,San-Miguel J,Ludwig H,Palumbo A,Obreja M,Aggarwal S,Moreau P

更新日期：2017-05-01 00:00:00

abstract：:Multiple myeloma (MM) is a disease with known immune dysregulation. Natural killer (NK) cells have shown preclinical activity in MM. We conducted a first-in-human study of umbilical cord blood-derived (CB) NK cells for MM patients undergoing high dose chemotherapy and autologous haematopoietic stem cell transplantatio...

journal_title：British journal of haematology

authors： Shah N,Li L,McCarty J,Kaur I,Yvon E,Shaim H,Muftuoglu M,Liu E,Orlowski RZ,Cooper L,Lee D,Parmar S,Cao K,Sobieiski C,Saliba R,Hosing C,Ahmed S,Nieto Y,Bashir Q,Patel K,Bollard C,Qazilbash M,Champlin R,Rezvani

更新日期：2017-05-01 00:00:00

abstract：:The success of genetically engineered T cells that express chimeric antigen receptors (CARTs) has been a momentous step forward in harnessing the potent cancer fighting abilities of the immune system. The efficacy seen in relapsed/refractory (r/r) acute lymphoblastic leukaemia (ALL), not only by inducing remission, bu...

journal_title：British journal of haematology

authors： Orlowski RJ,Porter DL,Frey NV

更新日期：2017-04-01 00:00:00

abstract：:The identification of acquired CALR mutations in patients with essential thrombocythaemia (ET) or myelofibrosis (MF) has meant that disease-initiating mutations can now be detected in about 90% of all patients with a myeloproliferative neoplasm (MPN). Here, we show that only those CALR mutations that cause a +1 frames...

journal_title：British journal of haematology

authors： Martin S,Wright CM,Scott LM

更新日期：2017-04-01 00:00:00