Haemolytic anaemia due to glucose-6-phosphate dehydrogenase (G6PD) deficiency: demonstration of two new biochemical variants, G6PD Hamm and G6PD Tarsus.

abstract：:It has been proposed that iron accumulation may contribute to atherogenesis by increasing free radical formation and oxidative stress. Epidemiological studies in which the association of iron status with atherosclerosis was assessed raised conflicting results. To test whether genetic haemochromatosis is associated wit...

journal_title：British journal of haematology

authors： Franco RF,Zago MA,Trip MD,ten Cate H,van den Ende A,Prins MH,Kastelein JJ,Reitsma PH

更新日期：1998-09-01 00:00:00

abstract：:Mutations of the p53 tumour suppressor gene occur in 20% of chronic myeloid leukaemia (CML) patients in blastic crisis, but it is still uncertain whether this inactivation plays a role in the pathogenesis of blastic transformation or in maintaining the leukaemic proliferation in CML, as it does in several solid tumour...

journal_title：British journal of haematology

authors： Lanza F,Bi S,Moretti S,Castoldi G,Goldman JM

更新日期：1995-05-01 00:00:00

abstract：:The activities of glucose-6-phosphate dehydrogenase (D-glucose-6-phosphate: NADP oxidoreductase, G6PD), 6-phosphogluconate dehydrogenase (6-phospho-D-gluconate: NADP oxidoreductase, 6PGD), hexokinase (ATP: D-hexose 6-phosphotransferase, Hx), lactate dehydrogenase (D-lactate: NAD oxidoreductase, LDH). glutamate oxaloac...

journal_title：British journal of haematology

authors： Brok-Simoni F,Ashkenazi YE,Holtzman F,Ramot B

更新日期：1975-02-01 00:00:00

abstract：:We have previously shown that polymorphonuclear leucocytes (PMN) harvested from children with cancer and exposed to chemotherapy exhibit defective bactericidal activities against both Gram-positive and Gram-negative microorganisms as well as accelerated apoptosis. In this study, PMN from children with cancer were eval...

journal_title：British journal of haematology

authors： Lejeune M,Cantinieaux B,Harag S,Ferster A,Devalck C,Sariban E

更新日期：1999-09-01 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：:A novel human leukaemia cell line, designated TMD7, was established from blast cells of a patient with de novo acute myeloblastic leukaemia with trilineage myelodysplasia (AML/TLD). As seen in the original blast cells, TMD7 cells expressed CD7, CD13, CD33 and CD34 and showed an abnormal karyotype containing -5, -7, -8...

journal_title：British journal of haematology

authors： Tohda S,Sakano S,Ohsawa M,Murakami N,Nara N

更新日期：2002-05-01 00:00:00

abstract：:Juvenile myelomonocytic leukaemia (JMML) is a unique myeloproliferative disorder of early childhood. Frequently, mutations in NRAS, KRAS, PTPN11, NF1 or CBL are found in these patients. Monosomy 7 is the most common cytogenetic aberration. To identify submicroscopic genomic copy number alterations, 20 JMML samples wer...

journal_title：British journal of haematology

authors： Ripperger T,Tauscher M,Praulich I,Pabst B,Teigler-Schlegel A,Yeoh A,Göhring G,Schlegelberger B,Flotho C,Niemeyer CM,Steinemann D

更新日期：2011-10-01 00:00:00

abstract：:Erythroid colonies derived from the circulating early erythroid precursor (BFU-E) of a patient with congenital dyserythropoietic anaemia type I (CDA I) have been grown in plasma clot and studied by electron microscopy. The number of circulating BFU-E was in the normal range with a roughly normal appearance at the ligh...

journal_title：British journal of haematology

authors： Vainchenker W,Guichard J,Bouguet J,Breton-Gorius J

更新日期：1980-09-01 00:00:00

abstract：:The heterogeneity of pyruvate kinase (PK) deficiency associated with hereditary haemolytic anaemia is emphasized by studies of a kindred harbouring two distinct mutant forms of this enzyme, both of which were kinetically defective with markedly decreased affinities for the substrate, phosphoenolypyruvate. The two isoe...

journal_title：British journal of haematology

authors： Pagila DE,Gray GR,Growe GH,Valentine WN

更新日期：1976-09-01 00:00:00

abstract：:Epistaxis is a common problem amongst anti-coagulated patients. Application of first-aid principles can control the majority of bleeds. In our sample of 60 patients attending the anticoagulant clinic, 40% were unable to think of a single measure that would be helpful in controlling a nose bleed. 6 weeks after being gi...

journal_title：British journal of haematology

authors： Lavy J

更新日期：1996-10-01 00:00:00

abstract：:An oscillatory pattern of platelet agglutination-disagglutination in response to Ristocetin (R) at narrow concentration ranges was observed in citrated platelet rich plasma (PRP) of 10 patients with Glanzmann's thrombasthenia. The cyclic pattern decreased in intensity over time, was reproducible, and was not pH depend...

journal_title：British journal of haematology

authors： Chediak J,Telfer MC,Vander Laan B,Maxey B,Cohen I

更新日期：1979-09-01 00:00:00

abstract：:To explore the possible role of a residual or variant alphaIIbbeta3 integrin (alphaIIbbeta3) in thrombogenesis, we used a new ex vivo perfusion chamber model to examine blood from patients with different subtypes of Glanzmann's thrombasthenia (GT). Non-anticoagulated blood was perfused through capillaries coated with ...

journal_title：British journal of haematology

authors： Hainaud P,Brouland JP,André P,Simoneau G,Bal Dit Sollier C,Drouet L,Caen J,Bellucci S

更新日期：2002-12-01 00:00:00

abstract：:The feasibility of marrow cryopreservation for autologous bone marrow transplantation after 7 d in liquid culture was assessed in 10 leukaemic patients. A median of 0.17 x 10(8) nucleated cells/kg and 0.4 x 10(4) CFU-GM/kg could be collected after the complete procedure, with overall a consistent cell loss. Long-term ...

journal_title：British journal of haematology

authors： Stoppa AM,Maraninchi D,Lafage M,Mannoni P,Novakovitch G,Blaise D,Gaspard MH,Michel G,Carcassonne Y

更新日期：1989-08-01 00:00:00

abstract：:Up-regulation of Fas/APO-1 (CD95) on haematopoietic progenitors and Fas-mediated apoptosis have been suggested to occur in a possible pathological mechanism in some bone marrow failure syndromes. We examined the expression of Fas antigen and susceptibility to Fas-mediated suppression of donor-derived haematopoietic ce...

journal_title：British journal of haematology

authors： Saheki K,Fujimori Y,Takemoto Y,Kakishita E

更新日期：2000-05-01 00:00:00

abstract：:Beta2-glycoprotein I (beta2GPI) is an important target antigen for antiphospholipid antibodies (aPL) and thus beta2GPI polymorphisms may influence aPL production and the development of antiphospholipid syndrome. We have studied the relationship between the Val247Leu and Trp316Ser beta2GPI polymorphisms and the aPL sta...

journal_title：British journal of haematology

authors： Camilleri RS,Mackie IJ,Humphries SE,Machin SJ,Cohen H

更新日期：2003-03-01 00:00:00

abstract：:The incidence of alpha-thalassaemia in an Italian population has been determined by a survey of random cord bloods for the presence of Hb Bart's. 144 out of 4730 (3%) had detectable amounts of Hb Bart's. Furthermore, alpha-globin gene analysis of 100 random cord bloods showed that five out of 100 had the common type o...

journal_title：British journal of haematology

authors： Velati C,Sampietro M,Biassoni M,Cappellini MD,Wainscoat JS,Higgs DR,Fiorelli G

更新日期：1986-07-01 00:00:00

abstract：:Factor VIII (FVIII) inhibitors appear in 3-20% of haemophilia A patients after injection of FVIII concentrates. However, autoantibodies to FVIII are also reported in non-haemophiliacs. In these patients FVIII inhibitor disappears spontaneously or diminishes in response to immunosuppressive therapy. However, a few pati...

journal_title：British journal of haematology

authors： Sunagawa T,Uezu Y,Kadena K,Tokuyama K,Kinjo F,Saito A

更新日期：1999-03-01 00:00:00

abstract：:The peripheral T-cell neoplasms are a biologically and clinically heterogeneous group of rare disorders that result from clonal proliferation of mature post-thymic lymphocytes. Natural killer (NK) cell neoplasms are included in this group. The World Health Organization classification of haemopoietic malignancies has d...

journal_title：British journal of haematology

authors： Dearden CE,Johnson R,Pettengell R,Devereux S,Cwynarski K,Whittaker S,McMillan A,British Committee for Standards in Haematology.

更新日期：2011-05-01 00:00:00

abstract：:The Kidd (JK) blood group system is of importance in transfusion medicine. The Jk(null) phenotype is associated with absence of the urea transporter in erythrocytes and moderately reduced ability to concentrate urine. We and others recently reported different molecular alterations in the silenced Jkb-like alleles of P...

journal_title：British journal of haematology

authors： Irshaid NM,Eicher NI,Hustinx H,Poole J,Olsson ML

更新日期：2002-02-01 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

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：:Nine patients with Fanconi anaemia (FA) were conditioned for HLA-identical sibling bone marrow transplant (BMT) with reduced dose of cyclophosphamide (Cy) without radiation or antithymocyte globulin (ATG). The total dose of Cy was 140 mg/kg (n = 2) or 120 mg/kg (n = 7). The median patient age was 8 years (range 4-19)....

journal_title：British journal of haematology

authors： Flowers ME,Zanis J,Pasquini R,Deeg HJ,Ribeiro R,Longton G,Medeiros CR,Doney K,Sanders J,Bryant J

更新日期：1996-03-01 00:00:00

abstract：:Thrombosis occurs in 37% of children with acute lymphoblastic leukaemia (ALL) and is related to an L-asparaginase-induced acquired antithrombin (AT) deficiency. The incidence dictates the need for anticoagulant prophylaxis. Direct thrombin inhibitors (DTI) are independent of AT for effect and may thus have advantages ...

journal_title：British journal of haematology

authors： Kuhle S,Lau A,Bajzar L,Vegh P,Halton J,Cherrick I,Anderson R,Desai S,McCusker P,Wu J,Abshire T,Mahoney D,Mitchell L

更新日期：2006-09-01 00:00:00

abstract：:In a patient with Philadelphia chromosome-positive acute lymphoblastic leukaemia (ALL), a novel variant of the chimaeric BCR-ABL mRNA transcript was detected by reverse transcription polymerase chain reaction (RT-PCR). Sequencing revealed the novel transcript to be a chimaeric mRNA produced by fusion of the BCR exon 1...

journal_title：British journal of haematology

authors： Hirota M,Hidaka E,Ueno I,Ishikawa M,Asano N,Yamauchi K,Ishida F,Tozuka M,Katsuyama T

更新日期：2000-09-01 00:00:00

abstract：:We report 14 normal peripheral blood stem cell (PBSC) donors > or = 60 years of age who had cytokine mobilization followed by PBSC apheresis for allogeneic transplantation. Mobilization was achieved with filgrastim (6 microg/kg twice daily). Their median age was 63.5 years (range 60-77), and 43% had a positive medical...

journal_title：British journal of haematology

authors： Anderlini P,Przepiorka D,Lauppe J,Seong D,Giralt S,Champlin R,Körbling M

更新日期：1997-05-01 00:00:00

abstract：:Decreased bone formation plays an important role in the development of lytic lesions during the late stage of multiple myeloma (MM). Release of insulin-like growth factor binding protein-4 (IGFBP4) by tumour cells adjacent to bone may inhibit IGF-I-stimulated osteoblast growth and contribute to decreased bone formatio...

journal_title：British journal of haematology

authors： Feliers D,Woodruff K,Abboud S

更新日期：1999-03-01 00:00:00

abstract：:Heterozygosity for a C8524T transition in the protein C gene converting Ser270(TCG) to Leu(TTG) in the protease domain was identified in a family with venous thrombosis. The mutation was associated with parallel reduction in plasma levels of protein C anticoagulant activity and protein C antigen, which is consistent w...

journal_title：British journal of haematology

authors： Lind B,Koefoed P,Thorsen S

更新日期：2001-06-01 00:00:00

abstract：:A 69-year-old woman presented with splenic marginal zone lymphoma associated with acquired von Willebrand's disease (AVWD). Laboratory abnormalities included markedly decreased plasma levels of factor VIII coagulant (C) activity (VIII:C 28%), von Willebrand's factor (VWF) antigen (Ag) (vWF:Ag < 6%), and VWF ristocetin...

journal_title：British journal of haematology

authors： Tefferi A,Hanson CA,Kurtin PJ,Katzmann JA,Dalton RJ,Nichols WL

更新日期：1997-03-01 00:00:00

abstract：:t(11;18)(q21;q21) has been recognized as a characteristic chromosomal translocation in mucosa-associated lymphoid tissue (MALT)-type lymphoma, and recent studies have demonstrated that this translocation results in the chimaeric transcript of API2 (apoptosis inhibitor 2)-MALT1 (mucosa-associated lymphoid tissue lympho...

journal_title：British journal of haematology

authors： Yonezumi M,Suzuki R,Suzuki H,Yoshino T,Oshima K,Hosokawa Y,Asaka M,Morishima Y,Nakamura S,Seto M

更新日期：2001-12-01 00:00:00

abstract：:Tissue factor pathway inhibitor (TFPI) is released to circulating blood after intravenous (i.v.) and subcutaneous (s.c.) injections of heparins, and may thus contribute to the antithrombotic effect of heparins. We have recently shown that total TFPI activity, plasma free TFPI antigen, and heparin releasable TFPI were ...

journal_title：British journal of haematology

authors： Bendz B,Hansen JB,Andersen TO,Ostergaard P,Sandset PM

更新日期：1999-12-01 00:00:00