abstract：:Risk assessment for patients with sickle cell disease (SCD) remains challenging as it depends on an individual physician's experience and ability to integrate a variety of test results. We aimed to provide a new risk score that combines clinical, laboratory, and imaging data. In a prospective cohort of 600 adult patie...

journal_title：British journal of haematology

authors： Sachdev V,Tian X,Gu Y,Nichols J,Sidenko S,Li W,Beri A,Layne WA,Allen D,Wu CO,Thein SL

更新日期：2021-01-28 00:00:00

abstract：:Thrombotic events are common in patients with multiple myeloma (MM), smouldering myeloma (SM) and monoclonal gammopathy of undetermined significance (MGUS). Previous studies have indicated platelet hyperactivation as a feature of thrombotic risk in MM, but there is a dearth of data in MGUS. In the present study, multi...

journal_title：British journal of haematology

authors： O'Sullivan LR,Meade-Murphy G,Gilligan OM,Mykytiv V,Young PW,Cahill MR

更新日期：2021-01-01 00:00:00

abstract：:A previous dose-finding study has suggested that romiplostim is effective in patients with refractory aplastic anaemia (AA) and 10 µg/kg once weekly was recommended as a starting dose. In this Phase II/III, multicentre, open-label study, romiplostim was administered subcutaneously at a fixed dose of 10 µg/kg once week...

journal_title：British journal of haematology

authors： Jang JH,Tomiyama Y,Miyazaki K,Nagafuji K,Usuki K,Uoshima N,Fujisaki T,Kosugi H,Matsumura I,Sasaki K,Kizaki M,Sawa M,Hidaka M,Kobayashi N,Ichikawa S,Yonemura Y,Enokitani K,Matsuda A,Ozawa K,Mitani K,Lee JW,Nakao

更新日期：2021-01-01 00:00:00

abstract：:The standard of care for patients with acute promyelocytic leukaemia (APL) relapsing after front-line treatment with arsenic trioxide (ATO)-based regimens remains to be defined. A total of 67 patients who relapsed after receiving ATO-based up-front therapy and were also salvaged using an ATO-based regimen were evaluat...

journal_title：British journal of haematology

authors： Fouzia NA,Sharma V,Ganesan S,Palani HK,Balasundaram N,David S,Kulkarni UP,Korula A,Devasia AJ,Nair SC,Janet NB,Abraham A,Mani T,Lakshmanan J,Balasubramanian P,George B,Mathews V

更新日期：2021-01-01 00:00:00

abstract：:Multiple myeloma (MM) tumour cells evade host immunity through a variety of mechanisms, which may potentially include the programmed cell death ligand-1 (PD-L1):programmed cell death protein-1 (PD-1) axis. This interaction contributes to the immunosuppressive bone marrow (BM) microenvironment, ultimately leading to re...

journal_title：British journal of haematology

authors： Chen H,Li M,Ng N,Yu E,Bujarski S,Yin Z,Wen M,Hekmati T,Field D,Wang J,Nassir I,Yu J,Huang J,Daniely D,Wang CS,Xu N,Spektor TM,Berenson JR

更新日期：2020-12-20 00:00:00

abstract：:The Fanconi anaemia protein FANCD2 suppresses PPARƔ to maintain haematopoietic stem cell's (HSC) function; however, the underlying mechanism is not known. Here we show that FANCD2 acts in concert with the Notch target HES1 to suppress inflammation-induced PPARƔ in HSC maintenance. Loss of HES1 exacerbates FANCD2-KO HS...

journal_title：British journal of haematology

authors： Wu L,Li X,Lin Q,Chowdhury F,Mazumder MH,Du W

更新日期：2020-11-22 00:00:00

abstract：:Kidney iron deposition measured by R2* (magnetic resonance imaging) MRI is posited to result from tubular reabsorption of filtered haemoglobin due to intravascular haemolysis. In chronically transfused sickle cell disease (SCD), R2* is elevated and positively correlated with lactate dehydrogenase (LDH). To account for...

journal_title：British journal of haematology

authors： Denton CC,Detterich JA,Coates TD,Wood JC

更新日期：2020-11-20 00:00:00

abstract：:Eltrombopag has been added to first-line treatment of immune aplastic anaemia (AA), resulting in higher responses. We analysed marrow samples of AA patients who responded to immunosuppressive therapy (IST) alone or in combination with eltrombopag for the composition of the haematopoietic stem and progenitor cell (HSPC...

journal_title：British journal of haematology

authors： Quintino de Oliveira B,Catto LFB,Santana BAA,Tellechea MF,Scheucher PS,Scheinberg P,Calado RT

更新日期：2020-11-20 00:00:00

abstract：:Ibrutinib is associated with durable responses in patients with Waldenström macroglobulinaemia (WM). We hypothesized that response depth is predictive of progression-free survival (PFS) in WM patients treated with ibrutinib. Using landmark analyses, we evaluated response depth in two cohorts of WM patients treated wit...

journal_title：British journal of haematology

authors： Castillo JJ,Abeykoon JP,Gustine JN,Zanwar S,Mein K,Flynn CA,Demos MG,Guerrera ML,Kofides A,Liu X,Munshi M,Tsakmaklis N,King R,Yang G,Hunter ZR,Advani RH,Palomba ML,Ansell SM,Gertz MA,Kapoor P,Treon SP

更新日期：2020-11-18 00:00:00

abstract：:Preclinical data suggests anti-lymphoma potential for statins, metformin and cyclooxygenase-2 (COX-2) inhibitors. We performed a retrospective population-based study of all adults aged ≥66 years diagnosed with diffuse large B-cell lymphoma (DLBCL) or transformed lymphoma treated with a rituximab containing regimen, be...

journal_title：British journal of haematology

authors： Smyth L,Blunt DN,Gatov E,Nagamuthu C,Croxford R,Mozessohn L,Cheung MC

更新日期：2020-11-01 00:00:00

abstract：:Epstein-Barr virus-associated haemophagocytic lymphohistiocytosis (EBV-HLH) is one of the most common subtypes of secondary HLH. However, more than 30% of patients do not respond to traditional treatment. Here, we investigated the efficacy and safety of the L-DEP regimen as a salvage therapy for paediatric refractory ...

journal_title：British journal of haematology

authors： Zhao Y,Li Z,Zhang L,Lian H,Ma H,Wang D,Zhao X,Zhang Q,Wang T,Zhang R

更新日期：2020-11-01 00:00:00

abstract：:Bendamustine + rituximab (BR) is the current first-line standard-of-care for chronic lymphocytic leukaemia (CLL) in fit patients aged 66-70 years, whereas chlorambucil + CD20 antibody is recommended in older patients with co-morbidities. This retrospective real-world study investigated whether risk-adapted BR was safe...

journal_title：British journal of haematology

authors： Mattsson A,Sylvan SE,Asklid A,Wiggh J,Winqvist M,Lundin J,Mansouri L,Rosenquist R,Johansson H,Österborg A,Hansson L

更新日期：2020-11-01 00:00:00

abstract：:Critically ill patients with coronavirus disease 2019 (COVID-19) present with hypoxaemia and are mechanically ventilated to support gas exchange. We performed a retrospective, observational study of blood gas analyses (n = 3518) obtained from patients with COVID-19 to investigate changes in haemoglobin oxygen (Hb-O2 )...

journal_title：British journal of haematology

authors： Vogel DJ,Formenti F,Retter AJ,Vasques F,Camporota L

更新日期：2020-11-01 00:00:00

abstract：:Sixty years ago there was little interest in venous thromboembolism (VTE) in global medical circles, and the British were no different in this respect. And yet, from nowhere the clinical field has developed: now prevention and management of VTE accounts for a large part of many consultant haematologists' working life,...

journal_title：British journal of haematology

authors： Hunt BJ

更新日期：2020-11-01 00:00:00

abstract：:This is an historical account of the randomised trials in chronic lymphocytic leukaemia in the UK between the years 1978 to 2004, describing their gestation, the treatments used and the main lessons learnt. Those lessons include: (1) how best to use chlorambucil, which was the first effective treatment for CLL; (2) th...

journal_title：British journal of haematology

authors： Catovsky D,Else M

更新日期：2020-11-01 00:00:00

abstract：:Patients with sickle cell disease (SCD) with high fetal haemoglobin (HbF) tend to have a lower incidence of complications and longer survival due to inhibition of deoxyhaemoglobin S (HbS) polymerisation by HbF. HbF-containing cells, namely F cells, are strongly influenced by genetic factors. We measured the percentage...

journal_title：British journal of haematology

authors： Urio F,Nkya S,Rooks H,Mgaya JA,Masamu U,Zozimus Sangeda R,Mmbando BP,Brumat M,Mselle T,Menzel S,Luzzatto L,Makani J

更新日期：2020-10-19 00:00:00

abstract：:Myelofibrosis is an enigmatic myeloproliferative neoplasm, despite noteworthy strides in understanding its genetic underpinnings. Driver mutations involving JAK2, CALR or MPL in 90% of patients mediate constitutive JAK-STAT signaling which, in concert with epigenetic alterations (ASXL1, DNMT3A, SRSF2, EZH2, IDH1/2 mut...

journal_title：British journal of haematology

authors： Gangat N,Tefferi A

更新日期：2020-10-01 00:00:00

abstract：:Metabolic tumour volume (MTV) and total lesion glycolysis (TLG) are positron-emission tomography/computed tomography (PET/CT) variables for predicting multiple myeloma's (MM) outcome. We retrospectively investigated and compared the predictive value of MTV, TLG and high-risk PET/CT variables in clinical practice in 18...

journal_title：British journal of haematology

authors： Terao T,Machida Y,Tsushima T,Miura D,Narita K,Kitadate A,Takeuchi M,Matsue K

更新日期：2020-10-01 00:00:00

abstract：:Minimal residual disease (MRD) in acute myeloid leukaemia (AML) poses a major challenge due to drug insensitivity and high risk of relapse. Intensification of chemotherapy and stem cell transplantation are often pivoted on MRD status. Relapse rates are high even with the integration of first-generation FMS-like tyrosi...

journal_title：British journal of haematology

authors： Zabkiewicz J,Lazenby M,Edwards G,Bygrave CA,Omidvar N,Zhuang L,Knapper S,Guy C,Hills RK,Burnett AK,Alvares CL

更新日期：2020-10-01 00:00:00

abstract：:Allogeneic haematopoietic stem cell transplantation (HSCT) after a reduced-intensity conditioning (RIC) regimen with fludarabine, melphalan and alemtuzmab is an effective therapy for haematological malignancies. Alemtuzumab, a monoclonal antibody against CD52, a glycosylphosphatidylinositol-anchor-bound surface protei...

journal_title：British journal of haematology

authors： Woelfinger P,Epp K,Schaefer L,Kriege D,Theobald M,Bopp T,Wagner-Drouet EM

更新日期：2020-10-01 00:00:00

abstract：:Standard treatment for classical Hodgkin lymphoma (cHL) is poorly tolerated in older patients and results disappointing. We assessed safety and efficacy of brentuximab vedotin (BV), in previously untreated patients with cHL unfit for standard treatment due to age, frailty or comorbidity. The primary outcome was comple...

journal_title：British journal of haematology

authors： Gibb A,Pirrie SJ,Linton K,Warbey V,Paterson K,Davies AJ,Collins GP,Menne T,McKay P,Fields PA,Miall FM,Nagy E,Wheatley K,Reed R,Baricevic-Jones I,Barrington S,Radford J

更新日期：2020-09-14 00:00:00

abstract：:In emicizumab prophylaxis, the concomitant therapy using bypassing agents (BPAs) is required for breakthrough bleeding and invasive procedures with attention to thrombotic complications. To predict coagulant effects of BPAs in emicizumab-treated patients with haemophilia A (PwHA) with inhibitor (PwHAwI), blood samples...

journal_title：British journal of haematology

authors： Furukawa S,Nogami K,Shimonishi N,Nakajima Y,Matsumoto T,Shima M

更新日期：2020-09-01 00:00:00

abstract：:Future progress in the treatment of multiple myeloma (MM) requires both the characterisation of key drivers of the disease and novel, innovative approaches to tackle these vulnerabilities. The present study focussed on the pre-clinical evaluation of a novel drug class, BMI-1 modulators, in MM. We demonstrate potent ac...

journal_title：British journal of haematology

authors： Bolomsky A,Muller J,Stangelberger K,Lejeune M,Duray E,Breid H,Vrancken L,Pfeiffer C,Hübl W,Willheim M,Weetall M,Branstrom A,Zojer N,Caers J,Ludwig H

更新日期：2020-09-01 00:00:00

abstract：:The Complement 1 trial investigated the efficacy and safety of ofatumumab + chlorambucil with chlorambucil monotherapy in patients with previously untreated chronic lymphocytic leukaemia (CLL). On long-term follow-up in the chemoimmunotherapy arm vs. the chemotherapy arm there was an estimated 12% (not significant) an...

journal_title：British journal of haematology

authors： Offner F,Robak T,Janssens A,Govind Babu K,Kloczko J,Grosicki S,Mayer J,Panagiotidis P,Schuh A,Pettitt A,Montillo M,Werner O,Vincent G,Khanna S,Hillmen P

更新日期：2020-09-01 00:00:00

abstract：:Leukaemic stem cells (LSC) have been experimentally defined as the leukaemia-propagating population and are thought to be the cellular reservoir of relapse in acute myeloid leukaemia (AML). Therefore, LSC measurements are warranted to facilitate accurate risk stratification. Previously, we published the composition of...

journal_title：British journal of haematology

authors： Hanekamp D,Snel AN,Kelder A,Scholten WJ,Khan N,Metzner M,Irno-Consalvo M,Sugita M,de Jong A,Oude Alink S,Eidhof H,Wilhelm M,Feuring-Buske M,Slomp J,van der Velden VHJ,Sonneveld E,Guzman M,Roboz GJ,Buccisano F,Vyas P

更新日期：2020-09-01 00:00:00

abstract：:The hepatitis C virus-positive (HCV+) mixed cryoglobulinaemia (MC) is associated with haematological alterations such as monoclonal B-cell lymphocytosis or non-Hodgkin lymphomas (NHLs). Antiviral therapy for MC, based on interferon and ribavirin, has been shown to be able to eliminate the viral replication as well as ...

journal_title：British journal of haematology

authors： Pozzato G,Mazzaro C,Artemova M,Abdurakhmanov D,Grassi G,Crosato I,Mauro E,Ghersetti M,Zorat F,Bomben R,Bulian P,Gattei V

更新日期：2020-08-13 00:00:00

abstract：:The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-associated coronavirus disease 2019 (COVID-19) is primarily manifested as a respiratory tract infection, but may affect and cause complications in multiple organ systems (cardiovascular, gastrointestinal, kidneys, haematopoietic and immune systems), whil...

journal_title：British journal of haematology

authors： Kastritis E,Wechalekar A,Schönland S,Sanchorawala V,Merlini G,Palladini G,Minnema M,Roussel M,Jaccard A,Hegenbart U,Kumar S,Cibeira MT,Blade J,Dimopoulos MA

更新日期：2020-08-01 00:00:00

abstract：:Many immune dysfunctions participate in immune thrombocytopenia (ITP) pathogenesis, including numeric and functional defects in suppressor T (Ts) cells and immune-regulation abnormalities in mesenchymal stem cells (MSCs). Recent studies showed that MSCs can promote Ts cell differentiation. Thus, we compared the Ts cel...

journal_title：British journal of haematology

authors： Li H,Guan Y,Sun B,Dou X,Liu X,Xue F,Fu R,Zhang L,Yang R

更新日期：2020-07-16 00:00:00

abstract：:Relapses involving the central nervous system (CNS) are rare in children and adolescents with ALK+ anaplastic large cell lymphoma (ALCL) treated with regimens including CNS prophylaxis. Early identification of patients at high-risk for CNS relapse would enable stratification and better adaptation of initial treatment ...

journal_title：British journal of haematology

authors： Del Baldo G,Abbas R,Woessmann W,Horibe K,Pillon M,Burke A,Beishuizen A,Rigaud C,Le Deley MC,Lamant L,Brugières L

更新日期：2020-07-09 00:00:00

abstract：:The level of venous thrombosis risk in women who experience spontaneous or induced pregnancy loss has previously been uncertain. However, recent data indicate that the risk of venous thrombosis in women undergoing pregnancy termination in the first trimester is increased two-fold compared to non-pregnant women but red...

journal_title：British journal of haematology

authors： Bagot CN,Pavord S,Hunt BJ,British Society of Haematology Obstetric Haematology Special Interest Group.

更新日期：2020-07-01 00:00:00

abstract：:Asparaginase (ASNase) is an important anti-leukaemic drug in the treatment of childhood acute lymphoblastic leukaemia (ALL) and non-Hodgkin lymphoma (NHL). A substantial proportion of patients develop hypersensitivity reactions with anti-ASNase neutralising antibodies, resulting in allergic reactions or silent inactiv...

journal_title：British journal of haematology

authors： Mondelaers V,Ferster A,Uyttebroeck A,Brichard B,van der Werff Ten Bosch J,Norga K,Francotte N,Piette C,Vandemeulebroecke K,Verbeke C,Schmidt S,Benoit Y,Lammens T,De Moerloose B

更新日期：2020-07-01 00:00:00

abstract：:Smouldering multiple myeloma (SMM) presents without MM defining symptoms. We aimed to identify patients with SMM with an 80% risk of progression within 2 years using only serum parameters. In total, 527 patients with SMM were included and divided into a training group (287 patients from the Czech Myeloma Group [CMG]) ...

journal_title：British journal of haematology

authors： Hájek R,Sandecka V,Špička I,Raab M,Goldschmidt H,Beck S,Minařík J,Pavlíček P,Radocha J,Heindorfer A,Jelínek T,Stejskal L,Brožová L,Ševčíková S,Straub J,Pika T,Pour L,Maisnar V,Seckinger A,Hose D

更新日期：2020-07-01 00:00:00

abstract：:In 2019 the UK Myeloma Research Alliance introduced the Myeloma Risk Profile (MRP) for prediction of outcome in patients with newly diagnosed multiple myeloma (MM), ineligible for autologous stem cell transplantation. To validate the MRP in a population-based setting we performed a study of the entire cohort of transp...

journal_title：British journal of haematology

authors： Redder L,Klausen TW,Vangsted AJ,Gregersen H,Andersen NF,Pedersen RS,Szabo AG,Frederiksen M,Frølund UC,Helleberg C,Nielsen LK,Pedersen PT,Salomo M,Gimsing P,Frederiksen H,Abildgaard N

更新日期：2020-06-09 00:00:00

abstract：:Neurological emergencies are frequently catastrophic events in the course of haematological malignancies (HM) that, if not promptly recognized and treated, may lead to lethal outcomes or chronic sequelae. They may occur at any time during the disease course, but are more frequently observed following relapse. Practice...

journal_title：British journal of haematology

authors： de la Fuente MI,Alderuccio JP,Lossos IS

更新日期：2020-06-01 00:00:00

abstract：:Polycythaemia vera (PV) patients have an overall comparatively favourable prognosis, but disease progression is very heterogeneous and life-threatening thrombosis and bleedings are frequent complications in untreated disease. Moreover, transformation to more severe secondary myelofibrosis and acute myeloid leukaemia c...

journal_title：British journal of haematology

authors： Andréasson B,Pettersson H,Wasslavik C,Johansson P,Palmqvist L,Asp J

更新日期：2020-06-01 00:00:00

abstract：:Clonal heterogeneity in multisited or recurrent lymphoid neoplasms is a phenomenon that has been increasingly studied in recent years. However, in mucosa-associated lymphoid tissue (MALT) lymphomas it remains largely unexplored. Patients diagnosed at our institution with multisited MALT lymphoma, from January 2009 to ...

journal_title：British journal of haematology

authors： Condom M,Climent F,Fernández D,Colomer D,Lopez-Guerra M,Varela M,Carro I,Maluquer C,Mercadal S,Oliveira AC,Pané M,Matias-Guiu X,González-Barca E,Sureda A,Domingo-Domenech E

更新日期：2020-05-23 00:00:00

abstract：:Follicular lymphoma (FL) is a heterogeneous disease; therefore, reliable prognostic tools are needed to plan treatment strategies. The FL International Prognostic Index (FLIPI) was developed before the rituximab era, while the PRIMA-PI was built on rituximab chemotherapy. Our objective was to evaluate these two progno...

journal_title：British journal of haematology

authors： Kimby E,Lockmer S,Holte H,Hagberg H,Wahlin BE,Brown P,Østenstad B

更新日期：2020-05-14 00:00:00

abstract：:Follicular lymphoma (FL) constitutes a significant proportion of lymphomas and shows frequent relapses. Beyond conventional chemotherapy, new therapeutic approaches have emerged, focussing on the interplay between lymphoma cells and the microenvironment. Here we report the immunophenotypic investigation of the microen...

journal_title：British journal of haematology

authors： Menter T,Tzankov A,Zucca E,Kimby E,Hultdin M,Sundström C,Beiske K,Cogliatti S,Banz Y,Cathomas G,Karjalainen-Lindsberg ML,Grobholz R,Mazzucchelli L,Sander B,Hawle H,Hayoz S,Dirnhofer S

更新日期：2020-05-01 00:00:00

abstract：:Non-endemic Burkitt lymphoma (BL) is a rare germinal centre B-cell-derived malignancy with the genetic hallmark of MYC gene translocation and with rapid tumour growth as a distinct clinical feature. To investigate treatment outcomes, loss of lifetime and relapse risk in adult BL patients treated with intensive immunoc...

journal_title：British journal of haematology

authors： Jakobsen LH,Ellin F,Smeland KB,Wästerlid T,Christensen JH,Jørgensen JM,Josefsson PL,Øvlisen AK,Holte H,Blaker YN,Grauslund JH,Bjørn J,Molin D,Lagerlöf I,Smedby KE,Colvin K,Thanarajasingam G,Maurer MJ,Habermann TM,So

更新日期：2020-05-01 00:00:00

abstract：:The combined incidence of classical Philadelphia-negative myeloproliferative neoplasm (MPN) is 6-9/100 000 with a peak frequency between 50 and 70 years. MPN is less frequent in women of reproductive age. However, for essential thrombocythaemia (ET) in particular there is a second peak in women of reproductive age and...

journal_title：British journal of haematology

authors： Robinson SE,Harrison CN

更新日期：2020-05-01 00:00:00