Bone marrow cells from patients with Shwachman-Diamond syndrome abnormally express genes involved in ribosome biogenesis and RNA processing.

abstract：:Guidelines on hereditary spherocytosis (HS) published in 2004 (Bolton-Maggs et al, 2004) are here replaced to reflect changes in current opinion on the surgical management, (particularly the indications for concomitant splenectomy with cholecystectomy in children with mild HS, and concomitant cholecystectomy with sple...

journal_title：British journal of haematology

authors： Bolton-Maggs PH,Langer JC,Iolascon A,Tittensor P,King MJ,General Haematology Task Force of the British Committee for Standards in Haematology.

更新日期：2012-01-01 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：: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：:Radiolabelled antiCD-20 antibodies have demonstrated single agent activity in relapsed diffuse large B-cell lymphoma (DLBCL). The S0433 clinical trial enrolled patients with newly diagnosed, advanced stage or bulky stage II, histologically confirmed DLBCL. Patients received six cycles of R-CHOP (rituximab, cyclophosph...

journal_title：British journal of haematology

authors： Friedberg JW,Unger JM,Burack WR,Gopal AK,Raju RN,Nademanee AP,Kaminski MS,Li H,Press OW,Miller TP,Fisher RI

更新日期：2014-08-01 00:00:00

abstract：:During definitive erythropoiesis, erythroid precursors undergo differentiation through multiple nucleated states to an enucleated reticulocyte, which loses its residual RNA/organelles to become a mature erythrocyte. Over the course of these transformations, continuous changes in membrane proteins occur, including shif...

journal_title：British journal of haematology

authors： Kodippili GC,Spector J,Kang GE,Liu H,Wickrema A,Ritchie K,Low PS

更新日期：2010-09-01 00:00:00

abstract：:Haemophagocytic lymphohistiocytosis (HLH) may cause meningoencephalitis and significant neurological sequelae. We examined the relationship between neurological symptoms and cerebrospinal fluid (CSF) at diagnosis, and long-term outcome, in all children enroled in the HLH-94-study prior to July 1, 2003, for whom inform...

journal_title：British journal of haematology

authors： Horne A,Trottestam H,Aricò M,Egeler RM,Filipovich AH,Gadner H,Imashuku S,Ladisch S,Webb D,Janka G,Henter JI,Histiocyte Society.

更新日期：2008-02-01 00:00:00

abstract：:The International Extranodal Lymphoma Study Group (IELSG) promoted this study to determine the inter-observer agreement in the application of the Groupe d' Etude des Lymphomes de l' Adulte (GELA) histological scoring system for evaluating residual disease in post-treatment gastric biopsies of patients with gastric Muc...

journal_title：British journal of haematology

authors： Copie-Bergman C,Wotherspoon AC,Capella C,Motta T,Pedrinis E,Pileri SA,Bertoni F,Conconi A,Zucca E,Ponzoni M,Ferreri AJ

更新日期：2013-01-01 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 pulmonary function tests (PFTs) with cardiac, pancreatic and liver iron in 76 thalassemia major (TM) patients. Restrictive lung disease was observed in 16%, hyperinflation in 32% and abnormal diffusing capacity in 3%. While no patients met Global Initiative for Chronic Lung Disease criteria for air...

journal_title：British journal of haematology

authors： Sohn EY,Noetzli LJ,Gera A,Kato R,Coates TD,Harmatz P,Keens TG,Wood JC

更新日期：2011-10-01 00:00:00

abstract：:Primary ocular lymphoma (POL), a lymphoma of the globe, is a restricted form of primary central nervous system lymphoma (PCNSL) that often progresses to the brain and meninges; frequently it is misdiagnosed until central nervous system (CNS) lymphoma develops. The optimal treatment has not yet been identified. We retr...

journal_title：British journal of haematology

authors： Hormigo A,Abrey L,Heinemann MH,DeAngelis LM

更新日期：2004-07-01 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：:An anomalous organization of the cytoskeleton has been described in lymphocytes from chronic lymphatic leukaemia and in only few cell lines. We have now studied normal and neoplastic lymphocytes and lymphoid cell lines of both T and B lineage in order to detect morphological differences in the expression of microfilam...

journal_title：British journal of haematology

authors： Zauli D,Gobbi M,Crespi C,Tazzari PL,Miserocchi F,Tassinari A

更新日期：1988-04-01 00:00:00

abstract：:We have investigated the potency of the C677T mutation in the methylene tetrahydrofolate reductase (MTHFR) gene as a genetic risk factor in women with a history of early (12 weeks gestation) recurrent miscarriage (three or more consecutive pregnancy losses). Fifty-seven of the tota...

journal_title：British journal of haematology

authors： Holmes ZR,Regan L,Chilcott I,Cohen H

更新日期：1999-04-01 00:00:00

abstract：:Myeloablative conditioning prior to allogeneic stem cell transplantation causes a rapid increase in transferrin saturation and potentially toxic non-transferrin-bound iron (NTBI) in plasma. We have studied the ability of repeatedly administered apotransferrin to maintain this iron in a transferrin-bound form. Twenty a...

journal_title：British journal of haematology

authors： Parkkinen J,Sahlstedt L,von Bonsdorff L,Salo H,Ebeling F,Ruutu T

更新日期：2006-10-01 00:00:00

abstract：:We present the rare occurrence of an inhibitor of factor VIII procoagulant arising in a patient with mild haemophilia A and rheumatoid arthritis. The inhibitor was transient and behaved like a low titre, type II factor VIII procoagulant inhibitor similar to previously reported cases (Biggs et al, 1972b). In vitro stud...

journal_title：British journal of haematology

authors： Bovill EG,Burns SL,Golden EA

更新日期：1985-10-01 00:00:00

abstract：:Sideroblastic anaemia includes a heterogeneous group of rare conditions, characterized by decreased haem synthesis and mitochondrial iron overload, which are diagnosed by the presence of ringed sideroblasts in the bone marrow aspirate. The most frequent form is X-linked sideroblastic anaemia, caused by mutations of de...

journal_title：British journal of haematology

authors： Camaschella C

更新日期：2008-10-01 00:00:00

abstract：:Rats were rendered anaemic by a single bleeding or by a single injection of phenylhydrazine. At various times after the onset of anaemia they were nephrectomized and challenged with a 6 h exposure to hypoxia. The erythropoietin titre observed at the end of this hypoxic period was corrected for renal erythropoietin ind...

journal_title：British journal of haematology

authors： Erslev AJ,Caro J,Kansu E,Silver R

更新日期：1980-05-01 00:00:00

abstract：:Dickkopf-1 (DKK1), expressed by myeloma cells, suppresses osteoblast function and plays a key role in bone disease in multiple myeloma. BHQ880, a human neutralizing IgG1 anti-DKK1 monoclonal antibody, is being investigated for its impact on multiple myeloma-related bone disease and as an agent with potential anti-myel...

journal_title：British journal of haematology

authors： Iyer SP,Beck JT,Stewart AK,Shah J,Kelly KR,Isaacs R,Bilic S,Sen S,Munshi NC

更新日期：2014-11-01 00:00:00

abstract：:Mantle cell lymphoma (MCL) is a heterogenic non-Hodgkin lymphoma entity, with a median survival of about 5 years. In 2008 we reported the early - based on the median observation time of 4 years - results of the Nordic Lymphoma Group MCL2 study of frontline intensive induction immunochemotherapy and autologous stem cel...

journal_title：British journal of haematology

authors： Geisler CH,Kolstad A,Laurell A,Jerkeman M,Räty R,Andersen NS,Pedersen LB,Eriksson M,Nordström M,Kimby E,Bentzen H,Kuittinen O,Lauritzsen GF,Nilsson-Ehle H,Ralfkiaer E,Ehinger M,Sundström C,Delabie J,Karjalainen-Lindsb

更新日期：2012-08-01 00:00:00

abstract：:Chronic ITP is due to antibody-induced destruction of platelets by the reticuloendothelial (RE) system. The role of complement in this process is unclear. We measured platelet-associated complement (PAC) components C3, C3bi, C4 and C9 in 16 patients with chronic ITP, in two of these patients prior to and after splenec...

journal_title：British journal of haematology

authors： Kurata Y,Curd JG,Tamerius JD,McMillan R

更新日期：1985-08-01 00:00:00

abstract：:Significant increases in prolyl hydroxylase activity, a key enzyme in the collagen biosynthetic pathway, were noted in the hepatic homogenates of iron-loaded animals as compared to controls. The increase in prolyl hydroxylase activity was seen without any light microscopic histologic evidence of cell necrosis or accum...

journal_title：British journal of haematology

authors： Weintraub LR,Goral A,Grasso J,Franzblau C,Sullivan A,Sullivan S

更新日期：1985-02-01 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：:We studied the trends of the Epstein-Barr virus (EBV) positivity rate in Hodgkin's disease (HD) between 1955 and 1999. The overall positivity rate gradually decreased over time [12 out of 23 (52%) in 1955-69; 18 out of 39 (46%) in 1970-84; 13 out of 44 (35%) in 1985-99; P = 0.135]. Interestingly, a marked decrease in ...

journal_title：British journal of haematology

authors： Takeuchi K,Morishita Y,Fukayama M,Mori S

更新日期：2001-05-01 00:00:00

abstract：:2-3-Dihydroxybenzoic acid was evaluated as a potentially useful, orally effective iron-chelating drug by performing iron balance studies in patients with beta-thalassaemia major. The administration of this substance at 25 mg/kg/d to five patients for 8 d caused an average increase in iron excretion of 4.5 mg/d. When t...

journal_title：British journal of haematology

authors： Peterson CM,Graziano JH,Grady RW,Jones RL,Vlassara HV,Canale VC,Miller DR,Cerami A

更新日期：1976-08-01 00:00:00

abstract：:The plasma concentrations of erythropoietin (Ep), soluble transferrin receptors (sTfRs), iron, total iron binding capacity (TIBC) and ferritin were monitored in five leukaemia patients undergoing autologous bone marrow stem cell transplantation (BMSCT) and in 10 lymphoma and 21 ovarian cancer patients undergoing autol...

journal_title：British journal of haematology

authors： Testa U,Rutella S,Martucci R,Scambia G,D'Onofrio G,Pierelli L,Sica S,Benedetti Panici PL,Menichella G,Foti E,Mastroberardino G,Mancuso S,Leone G,Peschle C

更新日期：1997-03-01 00:00:00

abstract：:Glucose-6-phosphate dehydrogenase (G6PD) deficient individuals are at increased risk of developing haemolysis following treatment with various antimalarial drugs. Reliable field tests for G6PD deficiency are thus needed in chemotherapy studies and their validity has to be assessed. In two phase II clinical trials on m...

journal_title：British journal of haematology

authors： Meissner PE,Coulibaly B,Mandi G,Mansmann U,Witte S,Schiek W,Müller O,Schirmer RH,Mockenhaupt FP,Bienzle U

更新日期：2005-11-01 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：:We detected two transversions in two unrelated Italian patients with type 2A von Willebrand disease (VWD): a C to A at nucleotide 8821 and a T to A at nucleotide 8830, resulting in the missense mutations Pro864His and Val867Glu respectively. Both mutations were in the heterozygous form and abolished the BstXI restrict...

journal_title：British journal of haematology

authors： Bernardi F,Casonato A,Marchetti G,Gemmati D,Bizzaro N,Pontara E,Girolami A

更新日期：1998-12-01 00:00:00

abstract：:There are considerable differences in haemopoietic activity between young children and adults on the one hand, and between adults and the elderly on the other. A fundamental unanswered question is whether these differences relate to discrete stages or are part of a continuous process. We have sought to define aspects ...

journal_title：British journal of haematology

authors： Marley SB,Lewis JL,Davidson RJ,Roberts IA,Dokal I,Goldman JM,Gordon MY

更新日期：1999-07-01 00:00:00

abstract：:We have identified a tissue kallikrein in polymorphonuclear (PMN) leucocytes of normal human blood and bone marrow by immunocytochemistry, radioimmunoassay and enzymology. Immunoreactive tissue kallikrein was visualized in the mature neutrophil leucocytes and in immature forms such as metamyelocytes and myelocytes. No...

journal_title：British journal of haematology

authors： Figueroa CD,MacIver AG,Bhoola KD

更新日期：1989-07-01 00:00:00