abstract：:T-cell large granular lymphocytic leukaemia (T-LGLL) is a chronic clonal lymphoproliferative disorder of cytotoxic T lymphocytes which commonly occurs in older patients and is often associated with autoimmune diseases. Among 246 patients with T-LGLL seen at our institution over the last 10 years, we encountered 15 cas...

journal_title：British journal of haematology

authors： Awada H,Mahfouz RZ,Durrani J,Kishtagari A,Jagadeesh D,Lichtin AE,Hill BT,Hamilton BK,Carraway HE,Nazha A,Majhail NS,Sobecks R,Visconte V,Kalaycio M,Sekeres MA,Maciejewski JP

更新日期：2020-04-01 00:00:00

abstract：:Mucormycosis has emerged as an increasingly important cause of morbidity and mortality in immunocompromised patients, but contemporary data in children are lacking. We conducted a nationwide multicentre study to investigate the characteristics of mucormycosis in children with haematological malignancies. The cohort in...

journal_title：British journal of haematology

authors： Elitzur S,Arad-Cohen N,Barg A,Litichever N,Bielorai B,Elhasid R,Fischer S,Fruchtman Y,Gilad G,Kapelushnik J,Kharit M,Konen O,Laor R,Levy I,Raviv D,Shachor-Meyouhas Y,Shvartser-Beryozkin Y,Toren A,Yaniv I,Nirel R,I

更新日期：2020-04-01 00:00:00

abstract：:In myeloproliferative neoplasms (MPN), JAK2V617F allele burden measurement has an impact on prognosis that helps in patient monitoring. Less is known about its usefulness in CALR-mutated cases. Additional mutations found by next-generation sequencing have also shown an impact on prognosis that may drive therapeutic ch...

journal_title：British journal of haematology

authors： Cottin L,Riou J,Orvain C,Ianotto JC,Boyer F,Renard M,Truchan-Graczyk M,Murati A,Jouanneau-Courville R,Allangba O,Mansier O,Burroni B,Rousselet MC,Quintin-Roué I,Martin A,Sadot-Lebouvier S,Delneste Y,Chrétien JM,Hunaul

更新日期：2020-03-01 00:00:00

abstract：:We explored the prognostic factors for children with very high-risk (VHR) Philadelphia chromosome (Ph) negative B-cell acute lymphoblastic leukaemia (B-ALL) and compared the therapeutic effects of intensive chemotherapy and unmanipulated haploidentical haematopoietic stem cell transplantation (haplo-HSCT) as post-remi...

journal_title：British journal of haematology

authors： Xue YJ,Suo P,Huang XJ,Lu AD,Wang Y,Zuo YX,Yan CH,Wu J,Kong J,Zhang XH,Chen YH,Jia YP,Liu KY,Han W,Xu LP,Zhang LP,Cheng YF

更新日期：2020-03-01 00:00:00

abstract：:Mastocytosis is a rare disease with varied presentation, myriad symptomatology and variable prognosis. Most patients present with cutaneous disease and mediator-related symptomatology with a small subset having systemic disease (systemic mastocytosis, SM). A subset of the latter develops synchronous or metachronous ha...

journal_title：British journal of haematology

authors： Radia DH,Green A,Oni C,Moonim M

更新日期：2020-03-01 00:00:00

abstract：:The impact of age, ethnicity and socio-economic deprivation in the era of novel anti-myeloma agents is unclear. Using linked national data from New Zealand, we evaluated the incidence, prevalence and overall survival (OS) of individuals who were diagnosed with myeloma between 2004 and 2016. The crude incidence rate in...

journal_title：British journal of haematology

authors： Chan HSH,Milne RJ

更新日期：2020-03-01 00:00:00

abstract：:Immunotherapies have emerged as highly promising approaches to treat cancer patients. Allogeneic haematopoietic cell transplantation (HCT) is the most validated tumour immunotherapy available to date but its clinical efficacy is limited by toxicities, such as graft-versus-host disease (GVHD) and treatment resistance l...

journal_title：British journal of haematology

authors： Paczesny S

更新日期：2020-02-10 00:00:00

abstract：:Acute graft-versus-host disease (GVHD) remains a major complication after allogeneic haematopoietic stem cell transplantation (allo-HSCT). The emergence of different immuno-prophylaxis strategies, such as post-transplant cyclophosphamide or anti-thymocyteglobulin has reduced the incidence of acute GVHD in recent years...

journal_title：British journal of haematology

authors： Zeiser R

更新日期：2019-12-01 00:00:00

abstract：:The treatment of Hodgkin lymphoma (HL) is one of early success. However, disease-free survival (DFS) does not reflect latent organ injury and its impact on health status and well-being beyond 5 years. In fact, we are at a crossroads, in terms of needing individualized approaches to maintain DFS, while minimizing late ...

journal_title：British journal of haematology

authors： Castellino SM,Parsons SK,Kelly KM

更新日期：2019-12-01 00:00:00

abstract：:Median overall survival (OS) has improved for patients with newly diagnosed multiple myeloma (NDMM), but prognosis varies depending on baseline patient characteristics. Current models use data from selected clinical trial populations, which prevent application to patients in an unselected community setting that reflec...

journal_title：British journal of haematology

authors： Terebelo HR,Abonour R,Gasparetto CJ,Toomey K,Durie BGM,Hardin JW,Jagannath S,Wagner L,Narang M,Flick ED,Srinivasan S,Yue L,Kitali A,Agarwal A,Rifkin RM,CONNECT MM Registry Investigators.

更新日期：2019-12-01 00:00:00

abstract：:Mutations in SOCS1 are frequent in primary mediastinal B-cell lymphoma and classical Hodgkin lymphoma. In the latter, SOCS1 mutations affect the length of the encoded protein (major mutations) and are associated with shorter patient survival. Two independent studies examined the prognostic impact of SOCS1 mutations in...

journal_title：British journal of haematology

authors： Mellert K,Martin M,Lennerz JK,Lüdeke M,Staiger AM,Kreuz M,Löffler M,Schmitz N,Trümper L,Feller AC,Hartmann S,Hansmann ML,Klapper W,Stein H,Rosenwald A,Ott G,Ziepert M,Möller P

更新日期：2019-12-01 00:00:00

abstract：:Bortezomib in combination with cyclophosphamide and dexamethasone (CyBorD, is a well-established frontline chemotherapy regimen for patients with multiple myeloma, but prospective data on elderly non-transplant eligible patients is limited. A total of 155 patients aged 70 years or older with newly diagnosed multiple m...

journal_title：British journal of haematology

authors： Chan H,Chai K,Shih S,Lewsey R,Chen K,McDiarmid B,Jackson S,Simpson D

更新日期：2019-11-01 00:00:00

abstract：:This study describes the use of a simple charcoal product (DOAC-RemoveTM ) to allow haemostasis assays on patients taking direct oral anticoagulants (DOAC). In the proposed algorithm, patients taking DOAC are screened using the dilute thrombin time (dTT) and anti-Xa assay. If either are positive then DOAC-Remove is ut...

journal_title：British journal of haematology

authors： Cox-Morton S,MacDonald S,Thomas W

更新日期：2019-11-01 00:00:00

abstract：:Simultaneous evaluation of coagulation and fibrinolysis facilitates an overall understanding of normal and pathological haemostasis. We established an assay for assessing clot formation and fibrinolysis simultaneously using clot waveform analysis by the trigger of a mixture of activated partial thromboplastin time rea...

journal_title：British journal of haematology

authors： Nogami K,Matsumoto T,Sasai K,Ogiwara K,Arai N,Shima M

更新日期：2019-11-01 00:00:00

abstract：:Ibrutinib is associated with response rate of 90% and median progression-free survival (PFS) in excess of 5 years in Waldenström macroglobulinaemia (WM) patients. CXCR4 mutations are detected in 30-40% of patients with WM and associate with lower rates of response and shorter PFS to ibrutinib therapy. Both frameshift ...

journal_title：British journal of haematology

authors： Castillo JJ,Xu L,Gustine JN,Keezer A,Meid K,Dubeau TE,Liu X,Demos MG,Kofides A,Tsakmaklis N,Chen JG,Munshi M,Guerrera ML,Chan GG,Patterson CJ,Yang G,Hunter ZR,Treon SP

更新日期：2019-11-01 00:00:00

abstract：:Graft-versus-host disease (GVHD) is a major cause of transplant-related mortality (TRM) after allogeneic haematopoietic stem cell transplantation (HSCT) and presents a challenge in haploidentical HSCT. GVHD may be prevented by ex vivo graft T-cell depletion or in vivo depletion of proliferating lymphocytes. However, b...

journal_title：British journal of haematology

authors： Roy DC,Lachance S,Cohen S,Delisle JS,Kiss T,Sauvageau G,Busque L,Ahmad I,Bernard L,Bambace N,Boumédine RS,Guertin MC,Rezvani K,Mielke S,Perreault C,Roy J

更新日期：2019-09-01 00:00:00

abstract：:Treatment response assessment in multiple myeloma (MM) relies on the detection of paraprotein in serum and/or urine, bone marrow morphology and immunohistochemistry. With remarkable advances in therapy, particularly in the newly diagnosed setting, achievement of complete remission became frequent, creating the need to...

journal_title：British journal of haematology

authors： Bal S,Weaver A,Cornell RF,Costa LJ

更新日期：2019-09-01 00:00:00

abstract：:Selinexor, an oral Selective Inhibitor of Nuclear Export, targets Exportin 1 (XPO1, also termed CRM1). Non-clinical studies support combining selinexor with proteasome inhibitors (PIs) and corticosteroids to overcome resistance in relapsed/refractory multiple myeloma (RRMM). We conducted a phase I dose-escalation tria...

journal_title：British journal of haematology

authors： Jakubowiak AJ,Jasielec JK,Rosenbaum CA,Cole CE,Chari A,Mikhael J,Nam J,McIver A,Severson E,Stephens LA,Tinari K,Rosebeck S,Zimmerman TM,Hycner T,Turowski A,Karrison T,Zonder JA

更新日期：2019-08-01 00:00:00

abstract：:MonoMAC is a complex primary immunodeficiency caused by mutations in the myeloid transcription factor GATA2, characterized by multilineage cytopenia with malignant complications and severe infections, including mycobacteria and herpesviruses. We describe the clinical presentation, genetics and antiviral inflammatory r...

journal_title：British journal of haematology

authors： Mardahl M,Jørgensen SE,Schneider A,Raaschou-Jensen K,Holm M,Veirum J,Kristensen TK,Johansen IS,Christiansen M,Assing K,Mogensen TH

更新日期：2019-08-01 00:00:00

abstract：:Molecular genetic studies of lymphoma have led to refinements in disease classification in the most recent World Health Organization update. Nevertheless, a 'one-size-fits-most' treatment strategy based on morphology remains widely used for lymphoma despite significant molecular heterogeneity within histopathologicall...

journal_title：British journal of haematology

authors： El-Galaly TC,Cheah CY,Villa D

更新日期：2019-08-01 00:00:00

abstract：:Allogeneic stem cell transplantation (alloSCT) is a curative procedure for myelofibrosis. Elderly people are mainly affected, limiting the feasibility of myeloablative regimens. The introduction of reduced-intensity conditioning (RIC) made alloSCT feasible for older patients. Nevertheless, the incidence of myelofibros...

journal_title：British journal of haematology

authors： Mannina D,Zabelina T,Wolschke C,Heinzelmann M,Triviai I,Christopeit M,Badbaran A,Bonmann S,von Pein UM,Janson D,Ayuk F,Kröger N

更新日期：2019-08-01 00:00:00

abstract：:Therapeutic plasma exchange (TPE) has been mainly used in the treatment of autoimmune diseases. The main mechanisms of action of TPE include the removal of circulating autoantibodies, immune complexes, complement components, cytokines and adhesion molecules, along with sensitization of antibody-producing cells to immu...

journal_title：British journal of haematology

authors： Zanatta E,Cozzi M,Marson P,Cozzi F

更新日期：2019-07-01 00:00:00

abstract：:Parental longevity is associated with an increased life expectancy; results with regard to specific diseases are conflicting. There are limited data focusing on host characteristics and their effect on survival among multiple myeloma (MM) patients and individuals with monoclonal gammopathy of undetermined significance...

journal_title：British journal of haematology

authors： Sverrisdóttir IS,Lund SH,Turesson I,Björkholm M,Goldin LR,Landgren O,Kristinsson SY

更新日期：2019-07-01 00:00:00

abstract：:Diamond-Blackfan anaemia (DBA) is a rare bone marrow failure syndrome characterised by anaemia, congenital anomalies and cancer predisposition. Although infections are the second leading cause of mortality in non-transplanted patients, immune function is largely unexplored. We identified quantitative deficits in serum...

journal_title：British journal of haematology

authors： Iskander D,Roberts I,Rees C,Szydlo R,Alikian M,Neale M,Harrington Y,Kelleher P,Karadimitris A,de la Fuente J

更新日期：2019-07-01 00:00:00

abstract：:Prior studies indicate that neutrophil extracellular traps (NETs) are associated with arterial thromboembolism (ATE) and mortality. We investigated the association between NET formation biomarkers (citrullinated histone H3 [H3Cit], cell-free DNA [cfDNA], and nucleosomes) and the risk of ATE and all-cause mortality in ...

journal_title：British journal of haematology

authors： Grilz E,Mauracher LM,Posch F,Königsbrügge O,Zöchbauer-Müller S,Marosi C,Lang I,Pabinger I,Ay C

更新日期：2019-07-01 00:00:00

abstract：:We retrospectively analysed 71 cases of Unicentric Castleman disease, a rare, usually asymptomatic, benign lymphoproliferative disorder presenting as a unique nodal mass. Although surgery is considered as the gold standard therapy, only 38 patients (54%) underwent initial surgical resection and 95% were cured. An addi...

journal_title：British journal of haematology

authors： Boutboul D,Fadlallah J,Chawki S,Fieschi C,Malphettes M,Dossier A,Gérard L,Mordant P,Meignin V,Oksenhendler E,Galicier L

更新日期：2019-07-01 00:00:00

abstract：:Paediatric non-Hodgkin lymphomas (pNHL) are a diverse group of malignancies characterised by nodal and/or extranodal involvement. Less common pNHL forms include those derived from mature T- and natural killer (NK) cells. Much of our current understanding of paediatric mature (non-anaplastic) T/NK-cell lymphomas with r...

journal_title：British journal of haematology

authors： Xavier AC,Suzuki R

更新日期：2019-06-01 00:00:00

abstract：:Lack of consensus for first-line marginal zone lymphoma (MZL) treatment and toxicities associated with currently available systemic therapies have inspired evaluation of immunotherapeutic agents yielding robust outcomes with improved tolerability. We previously reported durable efficacy with first-line lenalidomide an...

journal_title：British journal of haematology

authors： Becnel MR,Nastoupil LJ,Samaniego F,Davis RE,You MJ,Green M,Hagemeister FB,Fanale MA,Fayad LE,Westin JR,Wang M,Oki Y,Forbes SG,Feng L,Neelapu SS,Fowler NH

更新日期：2019-06-01 00:00:00

abstract：:In a prospective phase II trial, pentostatin combined with cyclophosphamide and rituximab (PCR) induced strong responses and was well-tolerated in previously untreated patients with advanced-stage, indolent non-Hodgkin lymphoma (iNHL). After a median patient follow-up of more than 108 months, we performed an intent-to...

journal_title：British journal of haematology

authors： Khashab T,Hagemeister F,Romaguera JE,Fanale MA,Pro B,McLaughlin P,Rodriguez MA,Neelapu SS,Fayad L,Younes A,Feng L,Vega F,Kwak LW,Samaniego F

更新日期：2019-05-01 00:00:00

abstract：:Mature (non-anaplastic) T-cell and natural killer (NK)-lymphomas rarely occur in children or adolescents. Due to the low incidence and heterogeneity, information regarding the aetiology, physiopathology and genetics of paediatric mature (non-anaplastic) T/NK-cell lymphoma is lacking. In addition, standard treatments h...

journal_title：British journal of haematology

authors： Flower A,Xavier AC,Cairo MS

更新日期：2019-05-01 00:00:00

abstract：:The use of immunochemotherapy has improved the outcome of follicular lymphoma (FL). Recently, complete response at 30 months (CR30) has been suggested as a surrogate for progression-free survival. This study aimed to analyse the life expectancy of FL patients according to their status at 30 months from the start of tr...

journal_title：British journal of haematology

authors： Magnano L,Alonso-Alvarez S,Alcoceba M,Rivas-Delgado A,Muntañola A,Nadeu F,Setoain X,Rodríguez S,Andrade-Campos M,Espinosa-Lara N,Rodríguez G,Sancho JM,Moreno M,Mercadal S,Carro I,Salar A,Garcia-Pallarols F,Arranz R,Ca

更新日期：2019-05-01 00:00:00

abstract：:Mantle cell lymphoma (MCL), a malignancy of B-lymphocytes, has a poor prognosis. It is thus necessary to improve the understanding of the pathobiology of MCL and identify factors contributing to its aggressiveness. Our studies, based on Affymetrix data from 17 MCL biopsies, real-time quantitative polymerase chain reac...

journal_title：British journal of haematology

authors： Mundt F,Merrien M,Nygren L,Sutton LA,Christensson B,Wahlin BE,Rosenquist R,Sander B,Wasik AM

更新日期：2019-05-01 00:00:00

abstract：:Sepsis may be associated with activation of the coagulation system and, in its most severe form, may result in disseminated intravascular coagulation (DIC). Initially, there is thrombosis primarily affecting small and medium sized vessels and contributing to organ dysfunction, but continued activation results in consu...

journal_title：British journal of haematology

authors： Scully M,Levi M

更新日期：2019-04-01 00:00:00

abstract：:Conflicting evidence exists on the epidemiology of type 2 diabetes mellitus (T2DM) among patients with sickle cell disease (SCD). This study measured the prevalence, incidence and clinical outcomes associated with T2DM in a large US population of commercially-insured adults aged ≥20 years with SCD between 2009 and 201...

journal_title：British journal of haematology

authors： Zhou J,Han J,Nutescu EA,Galanter WL,Walton SM,Gordeuk VR,Saraf SL,Calip GS

更新日期：2019-04-01 00:00:00

abstract：:The prognosis of paediatric acute myeloid leukaemia (AML) with primary induction failure (PIF) is extremely poor, and effective treatment strategies have not been established. We investigated the clinical and biological features of paediatric AML patients with PIF registered to the Japanese Paediatric Leukaemia/Lympho...

journal_title：British journal of haematology

authors： Miyamura T,Moritake H,Nakayama H,Tanaka S,Tomizawa D,Shiba N,Saito AM,Tawa A,Shimada A,Iwamoto S,Hayashi Y,Koike T,Horibe K,Manabe A,Mizutani S,Taga T,Adachi S

更新日期：2019-04-01 00:00:00

abstract：:Eculizumab is the first and only medication approved for paroxysmal nocturnal haemoglobinuria (PNH) and atypical haemolytic uraemic syndrome (aHUS) treatment. However, eculizumab safety based on long-term pharmacovigilance is unknown. This analysis summarises safety data collected from spontaneous and solicited source...

journal_title：British journal of haematology

authors： Socié G,Caby-Tosi MP,Marantz JL,Cole A,Bedrosian CL,Gasteyger C,Mujeebuddin A,Hillmen P,Vande Walle J,Haller H

更新日期：2019-04-01 00:00:00

abstract：:Hepatitis E virus (HEV) is increasingly found to cause hepatitis in allogeneic haematopoietic stem cell transplantation (HSCT) patients. However, little is known about HEV infection in patients receiving haploidentical HSCT (haplo-HSCT). Here, we retrospectively evaluate the incidence and clinical course of HEV infect...

journal_title：British journal of haematology

authors： Tang FF,Mo XD,Wang Y,Yan CH,Chen YH,Chen H,Han W,Chang YJ,Zhang HY,Xie YD,Ma H,Wei L,Xu LP,Huang XJ,Zhang XH

更新日期：2019-03-01 00:00:00

abstract：:Combined lenalidomide and dexamethasone is a standard-of-care therapy for the treatment of older adults with multiple myeloma. Lenalidomide monotherapy has not been evaluated in newly diagnosed myeloma patients. We conducted a phase II study, evaluating a response-adapted therapy for older adults newly diagnosed with ...

journal_title：British journal of haematology

authors： Baz R,Naqvi SMH,Lee JH,Brayer J,Hillgruber N,Fridley BL,Shain KH,Sullivan DM,Alsina M

更新日期：2019-03-01 00:00:00

abstract：:Our study aimed to determine the expression pattern and clinical relevance of CD99 in paediatric B-cell precursor acute lymphoblastic leukaemia (BCP-ALL). Our findings demonstrate that high expression levels of CD99 are mainly found in high-risk BCP-ALL, e.g. BCR-ABL1 and CRLF2Re/Hi, and that high CD99 mRNA levels are...

journal_title：British journal of haematology

authors： Chen D,Camponeschi A,Wu Q,Gerasimcik N,Li H,Shen X,Tan Y,Sjögren H,Nordlund J,Lönnerholm G,Abrahamsson J,Fogelstrand L,Mårtensson IL

更新日期：2019-02-01 00:00:00

abstract：:The Hodgkin lymphomas are a family of unique lymphoma subtypes, in which the nature of the neoplastic cell was enigmatic for many years. Much of the mystery has been solved, with all forms now considered to be of B-cell origin, in most cases of germinal centre derivation. Today we recognize Hodgkin lymphoma as an epon...

journal_title：British journal of haematology

authors： Wang HW,Balakrishna JP,Pittaluga S,Jaffe ES

更新日期：2019-01-01 00:00:00