Guidelines for the diagnosis and management of hereditary spherocytosis--2011 update.

abstract：:Tumour necrosis factor-α (TNF) is an inflammatory cytokine that is elevated in a number of autoimmune diseases including immune thrombocytopenia (ITP), a bleeding disorder characterized by low platelet counts. In vitro TNF blockade increases expansion of the regulatory T cell (Treg) IKZF2 (also termed Helios) subset i...

journal_title：British journal of haematology

authors： Zhong H,Bussel J,Yazdanbakhsh K

更新日期：2015-01-01 00:00:00

abstract：:As the cure rates for haematological malignancies have improved, the exploration of the balance between efficacy and side effects has become a major research target. The antifolate methotrexate is widely used in the treatment of acute lymphoblastic leukaemia, non-Hodgkin lymphoma, and osteosarcoma. Even when given ide...

journal_title：British journal of haematology

authors： Schmiegelow K

更新日期：2009-09-01 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：:In type 1 Gaucher disease a bleeding tendency occurs which is partly caused by thrombocytopenia due to massive splenomegaly. In addition, low levels of factors IX and XI have been described. The mechanism responsible for these clotting factor abnormalities is unknown. We performed a detailed study of parameters of coa...

journal_title：British journal of haematology

authors： Hollak CE,Levi M,Berends F,Aerts JM,van Oers MH

更新日期：1997-03-01 00:00:00

abstract：:Five paediatric cases of haemophagocytic lymphohistiocytosis (HLH) which showed proliferation of granular atypical lymphocytoid cells in bone marrow are reported. All cases were girls aged 8 months to 4 years who had marked hepatosplenomegaly. Marker analysis on peripheral blood mononuclear cells revealed an increase ...

journal_title：British journal of haematology

authors： Imashuku S,Hibi S,Morinaga S,Takagi K,Chen J,Mugishima H,Ishii T,Sako M,Arakawa H,Kato M

更新日期：1997-03-01 00:00:00

abstract：:Kinetic analysis of a single intravenous injection of 100 mg iron(III) hydroxide-sucrose complex (Venofer) mixed with 52Fe(III) hydroxide-sucrose as a tracer was followed for 3-6 h in four generally anaesthetized, artificially ventilated minipigs using positron emission tomography (PET). The amount of injected radioac...

journal_title：British journal of haematology

authors： Beshara S,Lundqvist H,Sundin J,Lubberink M,Tolmachev V,Valind S,Antoni G,Långström B,Danielson BG

更新日期：1999-02-01 00:00:00

abstract：:Atrial fibrillation (AF) is a common cardiac arrhythmia with a 5-20% annual risk of stroke. Warfarin reduces this risk by at least 60%. Despite adequate anticoagulation within the target International Normalized Ratio (INR) range of 2.0-3.0, some patients still experience thrombotic and bleeding events. It is now poss...

journal_title：British journal of haematology

authors： Gatt A,van Veen JJ,Bowyer A,Woolley AM,Cooper P,Kitchen S,Makris M

更新日期：2008-09-01 00:00:00

abstract：:Elevated levels of haemoglobin F (Hb F) have been foudn in a wide range of haematological malignancies, but very high levels were found only in juvenile chronic myeloid leukaemia (JCML), and erythroleukaemia occurring in infancy. In both these disorders a reversion to a fetal form of erythropoiesis may occur, as judge...

journal_title：British journal of haematology

authors： Sheridan BL,Weatherall DJ,Clegg JB,Pritchard J,Wood WG,Callender ST,Durrant IJ,McWhirter WR,Ali M,Partridge JW,Thompson EN

更新日期：1976-04-01 00:00:00

abstract：:With the aim of producing unique targets for malignant cells we have identified peptide ligands for the clonal surface immunoglobulin isolated from the B cells of a chronic lymphocytic leukaemia (CLL) patient. The peptides were identified from random-peptide phage-display libraries. The obtained ligands bound specific...

journal_title：British journal of haematology

authors： Buhl L,Szecsi PB,Gisselø GG,Schafer-Nielsen C

更新日期：2002-03-01 00:00:00

abstract：:The peripheral blood lymphoid cells of five patients with Sézary syndrome (SS) were examined with respect to their surface membrane characteristics and their response to mitogens. These cells showed markedly defective mitogenic responses to a broad dose range of phytohaemagglutinin (PHA), pokeweed mitogen, concanavali...

journal_title：British journal of haematology

authors： Braylan R,Variakojis D,Yachnin S

更新日期：1975-12-01 00:00:00

abstract：:The pathogenesis of HELLP (haemolysis, elevated liver enzyme and low platelet count) syndrome, a severe presentation of pre-eclampsia, is still an enigma. Activated protein C resistance resulting from a mutation in coagulation factor V has recently emerged as the leading cause of thrombosis in pregnancy. We report on ...

journal_title：British journal of haematology

authors： Brenner B,Lanir N,Thaler I

更新日期：1996-03-01 00:00:00

abstract：:Transient leukaemia (TL) in neonates with Down syndrome (DS) is characterized by the transient appearance of blast cells in the peripheral blood that resolves spontaneously. Some TL patients die at an early age due to organ failure. Seventy DS patients with TL were studied retrospectively to identify clinical and labo...

journal_title：British journal of haematology

authors： Muramatsu H,Kato K,Watanabe N,Matsumoto K,Nakamura T,Horikoshi Y,Mimaya J,Suzuki C,Hayakawa M,Kojima S

更新日期：2008-08-01 00:00:00

abstract：:We tested four negative and two positive selection methods for separation of CD34+ cells from mobilized blood cells, and analysed fold-enrichment, purity and recovery of CD34+ cells after selection procedures. The elimination of mature CD34- cells was achieved by adhesion to nylon-wool fibre (5.9 +/- 1.0 mean fold-enr...

journal_title：British journal of haematology

authors： Di Nicola M,Bregni M,Siena S,Ruffini PA,Milanesi M,Ravagnani F,Gianni AM

更新日期：1996-09-01 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：:Red cell lifespan has been measured using 51Cr and 59Fe in 19 patients. 59Fe can be used to give results which agree closely with those obtained using 51Cr provided that the plasma 59Fe clearance curve is properly defined and the data analysed correctly. In some patients elution of 51Cr may be more than three times th...

journal_title：British journal of haematology

authors： Ricketts C,Cavill I,Napier JA

更新日期：1977-11-01 00:00:00

abstract：:Immune reconstitution was studied prospectively in 66 children who underwent 77 haematopoietic cell transplantations (HCT): 46 autologous HCTs in 39 patients and 31 allogeneic HCTs in 27 patients. We studied the dynamic analysis of immune recovery with regard to potential factors affecting its speed, including age, ty...

journal_title：British journal of haematology

authors： Kalwak K,Gorczyńska E,Toporski J,Turkiewicz D,Slociak M,Ussowicz M,Latos-Grazyńska E,Król M,Boguslawska-Jaworska J,Chybicka A

更新日期：2002-07-01 00:00:00

abstract：:Major ABO mismatching is not considered a contraindication to allogeneic haematopoietic stem cell transplantation (HSCT). Modern reduced-intensity conditioning and reduced-toxicity regimens cause much less myeloablation than conventional myeloablative regimens, such as cyclophosphamide with busulfan or total body irra...

journal_title：British journal of haematology

authors： Aung FM,Lichtiger B,Bassett R,Liu P,Alousi A,Bashier Q,Ciurea SO,de Lima MJ,Hosing C,Kebriaei P,Nieto Y,Oran B,Parmar S,Qazilbash M,Shah N,Khouri I,Champlin RE,Popat U

更新日期：2013-03-01 00:00:00

abstract：:We report a therapy-related MDS (RAEB) patient with eosinophilia, unbalanced translocation der(7)t(1;7) (q12;q22) and lung cancer. We observed no increase in cytokine levels in serum or in the conditioned medium (CM) of peripheral T cells cultured with or without IL-2. When bone marrow (BM) cells were cultured with GM...

journal_title：British journal of haematology

authors： Imai Y,Yasuhara S,Hanafusa N,Ohsaka A,Enokihara H,Tomizuka H,Sonoyama M,Miura YS,Tohda S,Nara N,Takahashi A

更新日期：1996-12-01 00:00:00

abstract：:The determinants of steady state haemoglobin levels in sickle cell-haemoglobin C (SC) disease were investigated by measuring routine haematological and biochemical indices, red cell survival, oxygen affinity, pitted erythrocytes, and red cell and plasma volumes in 31 adult patients (15 male; 16 female). Red cell survi...

journal_title：British journal of haematology

authors： Bannerman RM,Serjeant B,Seakins M,England JM,Serjeant GR

更新日期：1979-09-01 00:00:00

abstract：:This study compared how Enoxaparin and unfractionated (UF) heparin influenced in vivo coagulation in patients randomized to receive, by twice daily subcutaneous injections, either 30 mg of Enoxaparin or 7500 I.U. of UF heparin after elective hip surgery. These two regimens were equally effective in reducing the incide...

journal_title：British journal of haematology

authors： Ofosu FA,Levine M,Craven S,Dewar L,Shafai S,Blajchman MA

更新日期：1992-10-01 00:00:00

abstract：:Endothelial cells (EC) were isolated from the umbilical vein of a newborn girl with type I 'platelet low' von Willebrand disease (I vWD) and endothelial localization and release and the ability of subendothelial von Willebrand factor (vWF) to support platelet adhesion were compared with those of normal EC. vWF was det...

journal_title：British journal of haematology

authors： Federici AB,de Groot PG,Moia M,Ijsseldijk MJ,Sixma JJ,Mannucci PM

更新日期：1993-01-01 00:00:00

abstract：:Besides circulating in normal plasma, von Willebrand factor (VWF) is also stored at relatively high concentration within the alpha-granules of platelets. This pool of platelet VWF exists distinct from plasma VWF, and is enriched in haemostatically-active high molecular weight multimers. Interestingly, the glycosylatio...

journal_title：British journal of haematology

authors： McGrath RT,McRae E,Smith OP,O'Donnell JS

更新日期：2010-03-01 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：: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：:We observed macrothrombocytopenia with leucocyte inclusions in 10 out of 14 members of a four-generation family. Morphological features of leucocyte inclusions and the presence of Alport-related symptoms supported the diagnosis of Fechtner syndrome. Compared to the two previously reported Fechtner families, our kindre...

journal_title：British journal of haematology

authors： Rocca B,Laghi F,Zini G,Maggiano N,Landolfi R

更新日期：1993-10-01 00:00:00

abstract：:The 'high and low responder phenomenon' of monocyte tissue factor (MTF) activity has been attributed to effects on monocytes by granulocytes, platelets and lipopolysaccharide (LPS). To study the possible contribution of plasma to the high and low responder phenomenon, we measured the MTF activity in isolated cryoprese...

journal_title：British journal of haematology

authors： Nijziel M,van Oerle R,van 't Veer C,van Pampus E,Lindhout T,Hamulyák K

更新日期：2001-01-01 00:00:00

abstract：:The purpose of the present work were to identify the initial characteristics associated with long-term survival in chronic granulocytic leukaemia (CGL) and to analyse the accuracy of prognostic models in identifying long-term survivors. 813 Philadelphia (Ph) chromosome-positive, nonblastic CGL patients from six Americ...

journal_title：British journal of haematology

authors： Cervantes F,Robertson JE,Rozman C,Baccarani M,Tura S,Gómez GA,Braun TJ,Clarkson BD,Pereira A

更新日期：1994-06-01 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：:Platelets from patients with platelet-type von Willebrand disease (vWD) were used as immunogens for the production of murine monoclonal antibodies (MoAbs). One such MoAb, C-34, inhibited ristocetin-induced aggregation of patient or normal platelets, but not aggregation induced by other aggregating agents. As demonstra...

journal_title：British journal of haematology

authors： Miller JL,Hustad KO,Kupinski JM,Lyle VA,Kunicki TJ

更新日期：1990-03-01 00:00:00

abstract：:Monoclonal gammopathy of undetermined significance (MGUS) has been associated with an increased risk of thrombosis. We carried out a retrospective multicentre cohort study on 1491 patients with MGUS. In 49 patients (3.3%) MGUS was diagnosed after a thrombotic event. Follow-up details for a period of at least 12 months...

journal_title：British journal of haematology

authors： Za T,De Stefano V,Rossi E,Petrucci MT,Andriani A,Annino L,Cimino G,Caravita T,Pisani F,Ciminello A,Torelli F,Villivà N,Bongarzoni V,Rago A,Betti S,Levi A,Felici S,Gentilini F,Calabrese E,Leone G,Multiple Myeloma G

更新日期：2013-03-01 00:00:00