Acquired Pseudoxanthoma elasticum-like syndrome in beta-thalassaemia patients.

abstract：:Diagnosing central nervous system (CNS) lymphoma remains a challenge. Most patients have to undergo brain biopsy to obtain tissue for diagnosis, with associated risks of serious complications. Diagnostic markers in blood or cerebrospinal fluid (CSF) could facilitate early diagnosis with low complication rates. We perf...

journal_title：British journal of haematology

authors： van Westrhenen A,Smidt LCA,Seute T,Nierkens S,Stork ACJ,Minnema MC,Snijders TJ

更新日期：2018-08-01 00:00:00

abstract：:Allele alphaLELY is a common low-expression allele of the erythroid spectrin SPTA1 gene. It results in the aggravated expression of hereditary elliptocytosis due to SPTA1 gene mutations occurring in trans. Allele alphaLELY contains, in particular, mutations in introns 45 and 46, both in polypyrimidine tracts, and caus...

journal_title：British journal of haematology

authors： Wilmotte R,Marechal J,Delaunay J

更新日期：1999-03-01 00:00:00

abstract：:The incidence, characteristics and risk factors for the development of osteonecrosis of the jaw (ONJ) were evaluated among 303 myeloma patients. Only patients who received bisphosphonates developed ONJ (28/254; 11%). Zoledronic acid produced 9.5-fold greater risk for developing ONJ than pamidronate alone (P = 0.042) a...

journal_title：British journal of haematology

authors： Zervas K,Verrou E,Teleioudis Z,Vahtsevanos K,Banti A,Mihou D,Krikelis D,Terpos E

更新日期：2006-09-01 00:00:00

abstract：:Blood from five donors, previously shown to be positive for cytomegalovirus (CMV) DNA following polymerase chain reaction (PCR) amplification, was filtered through commercially available leucocyte filters. Analysis of pre- and post-filtration samples by PCR with ethidium bromide staining has shown that filtration was ...

journal_title：British journal of haematology

authors： Smith KL,Cobain T,Dunstan RA

更新日期：1993-04-01 00:00:00

abstract：:We evaluated the performance of eight d-assays for the exclusion of deep vein thrombosis (DVT); Biopool AutoDimer, Biopool MiniQuant, bioMèrieux MDA D-Dimer, VIDAS, Dade Behring D-Dimer Plus, Trinity Biotech AMAX, NycoCard D-dimer and IL Test D-Dimer. The assays were evaluated both as stand-alone tests, and in combina...

journal_title：British journal of haematology

authors： Gardiner C,Pennaneac'h C,Walford C,Machin SJ,Mackie IJ

更新日期：2005-03-01 00:00:00

abstract：:The serum "uracil+uridine" level, expressed as uracil, has been measured in 21 cases of vitamin B12 deficiency, in which the serum folate was normal, and compared with the level in 97 normal subjects. The level in the vitamin B12 deficient group (11.9 mumol/1). was significantly lower than in the controls (15.7 mumol/...

journal_title：British journal of haematology

authors： Parry TE,Blackmore JA

更新日期：1976-12-01 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：:A 29-year-old man in remission from acute myeloblastic leukaemia was treated by chemoradiotherapy and transplantation of bone marrow (BMT) collected from his HLA identical brother. Engraftment was documented on D12. Transient acute GVHD (grade II) appeared from D34. No infection complicated the BMT. Nevertheless sever...

journal_title：British journal of haematology

authors： Bierling P,Cordonnier C,Fromont P,Rodet M,Tanzer J,Vernant JP,Bracq C,Duedari N

更新日期：1985-04-01 00:00:00

abstract：:Positron emission tomography using fluorine-18 (FDG-PET) is increasingly used in the staging and follow-up of malignant lymphomas, although its precise role has not yet been determined. This review considers the results reported at the different stages in the disease history and separately considers the major histolog...

journal_title：British journal of haematology

authors： Burton C,Ell P,Linch D

更新日期：2004-09-01 00:00:00

abstract：:Acquired haemophilia A (AHA) is a rare bleeding disorder characterized by the sudden generation of autoantibodies against factor VIII (FVIII) in individuals with no previous history of abnormal haemostasis. Understanding the pathogenesis of this disease has been hampered by the rarity of the patients and the difficult...

journal_title：British journal of haematology

authors： Mahendra A,Padiolleau-Lefevre S,Kaveri SV,Lacroix-Desmazes S

更新日期：2012-01-01 00:00:00

abstract：:Pleiotrophin (PTN), a tightly regulated angiogenic and mitogenic heparin-binding protein, is markedly elevated in a variety of aggressive solid tumours. The role of PTN in haematological malignancies, however, has not been previously evaluated. This study demonstrated that PTN serum levels were elevated in multiple my...

journal_title：British journal of haematology

authors： Yeh HS,Chen H,Manyak SJ,Swift RA,Campbell RA,Wang C,Li M,Lee HJ,Waterman G,Gordon MS,Ma J,Bonavida B,Berenson JR

更新日期：2006-06-01 00:00:00

abstract：:Overt ischaemic stroke is one of the most devastating complications in children with sickle cell disease (SCD). The compensatory response to anaemia in SCD includes an increase in cerebral blood flow (CBF) by accessing cerebrovascular dilatory reserve. Exhaustion of dilatory reserve secondary to anaemic stress may lea...

journal_title：British journal of haematology

authors： Kosinski PD,Croal PL,Leung J,Williams S,Odame I,Hare GM,Shroff M,Kassner A

更新日期：2017-01-01 00:00:00

abstract：:The use of 111Indium oxine as a platelet label for the performance of platelet life-span studies has been examined. Platelet life-span in normal subjects varied between 8 X 10 and 10 X 36 d. Patients with primary thrombocythaemia had clearly reduced platelet life-span whether or not they presented with vascular occlus...

journal_title：British journal of haematology

authors： Bautista AP,Buckler PW,Towler HM,Dawson AA,Bennett B

更新日期：1984-12-01 00:00:00

abstract：:In advanced chronic myeloid leukaemia patients, STI-571 produces complete haematological response in most cases and cytogenetic response in up to 50%. However, these patients often suffer periods of pancytopenia, which can lead to life-threatening complications, and is probably due to the small number of residual norm...

journal_title：British journal of haematology

authors： Lewalle P,Meuleman N,Verhest A,Bron D,Martiat P

更新日期：2002-07-01 00:00:00

abstract：:Heparin binding on polymorphonuclear leucocytes (PMNL) was characterized. Heparin binding was specific, rapid, saturable and reversible. One single class of heparin binding sites was found with a dissociation constant of 1.22 mumol/l and 7.7 x 10(6) sites per PMNL. The binding was independent of the anticoagulant acti...

journal_title：British journal of haematology

authors： Leculier C,Benzerara O,Couprie N,Francina A,Lasne Y,Archimbaud E,Fiere D

更新日期：1992-05-01 00:00:00

abstract：:Polycythaemia vera (PV) patients' blood burst-forming units-erythroid (BFU-E) have an enhanced sensitivity to stem cell factor (SCF) compared to normal BFU-E. To characterize SCF receptors on erythroid progenitors from normal individuals and PV patients, we performed binding experiments using radioiodinated recombinan...

journal_title：British journal of haematology

authors： Dai CH,Krantz SB,Koury ST,Kollar K

更新日期：1994-11-01 00:00:00

abstract：:Lack of reliable laboratory parameters is the main challenge in the management of fetal and neonatal alloimmune thrombocytopenia (FNAIT). Despite the long-known association between the HLA-DRB3*01:01 allele and human platelet antigen 1a (HPA-1a) alloimmunisation, maternal human leucocyte antigen (HLA) typing has been ...

journal_title：British journal of haematology

authors： Sainio S,Javela K,Tuimala J,Haimila K

更新日期：2017-01-01 00:00:00

abstract：:Cytometry has become important in the detection and determination of risk of monoclonal B-cell lymphocytosis; methodology has changed, and will continue to change, as cytometric technology changes. ...

journal_title：British journal of haematology

authors： Shapiro HM

更新日期：2007-12-01 00:00:00

abstract：:The prognostic significance of cytogenetic abnormalities was determined in 106 patients with well-characterized idiopathic myelofibrosis who were successfully karyotyped at diagnosis. 35% of the cases exhibited a clonal abnormality (37/106), whereas 65% (69/106) had a normal karyotype. Three characteristic defects, na...

journal_title：British journal of haematology

authors： Reilly JT,Snowden JA,Spearing RL,Fitzgerald PM,Jones N,Watmore A,Potter A

更新日期：1997-07-01 00:00:00

abstract：:In this study we have investigated the molecular basis for a mild form of beta-thalassaemia in three patients of Italian descent. In two, belonging to different families and affected by a mild and late-presenting form of thalassaemia major, direct sequencing of amplified DNA detected a C----T substitution at position ...

journal_title：British journal of haematology

authors： Meloni A,Rosatelli MC,Faà V,Sardu R,Saba L,Murru S,Sciarratta GV,Baldi M,Tannoia N,Vitucci A

更新日期：1992-02-01 00:00:00

abstract：:Eradication of Helicobacter pylori infection has been associated with the correction of thrombocytopenia in patients with idiopathic thrombocytopenic purpura (ITP). We have analysed the response to eradication of H. pylori in a series of 56 adult patients with chronic ITP. Forty patients had H. pylori infection (71%) ...

journal_title：British journal of haematology

authors： Jarque I,Andreu R,Llopis I,De la Rubia J,Gomis F,Senent L,Jiménez C,Martín G,Martínez JA,Sanz GF,Ponce J,Sanz MA

更新日期：2001-12-01 00:00:00

abstract：:This case study provides evidence for clonal evolution in pre-B-cell leukaemia. At diagnosis, the lymphoblasts from a 3-year-old boy were morphologically subtyped as L1 (French-American-British classification). Their immunophenotype was CALLA+, CIgM+, SIg-, TdT+, and the karyotype was pseudodiploid with a 1;19 translo...

journal_title：British journal of haematology

authors： Abromowitch M,Williams DL,Melvin SL,Stass S

更新日期：1984-03-01 00:00:00

abstract：:We studied the abnormal in vitro polyclonal B-cell activity observed in patients with multiple myeloma and Waldenström's macroglobulinaemia. Numbers of cells spontaneously secreting immunoglobulin (Ig) in freshly isolated suspensions of peripheral blood mononuclear cells and pokeweed mitogen (PWM) stimulated cultures ...

journal_title：British journal of haematology

authors： Levinson AI,Dziarski A,Blankenhorn E,Schreiber AD,Negendank WG

更新日期：1985-07-01 00:00:00

abstract：:AML1/RUNX1, which encodes a transcription factor essential for definitive haematopoiesis, is a frequent target of leukaemia-associated chromosome translocations. Point mutations of this gene have also recently been associated with leukaemia and myelodysplastic syndrome (MDS). To further define the frequency and biolog...

journal_title：British journal of haematology

authors： Nakao M,Horiike S,Fukushima-Nakase Y,Nishimura M,Fujita Y,Taniwaki M,Okuda T

更新日期：2004-06-01 00:00:00

abstract：:The existence of two genetic variants (allotypes) of normal human factor IX is used for carrier detection in three families with severe and one with mild haemophilia B. By analysis of IX:Ag with two different monoclonal antibodies in 93 members of the families, allelic assignment is shown to be a complement in carrier...

journal_title：British journal of haematology

authors： Wallmark A,Ljung R,Nilsson IM

更新日期：1987-12-01 00:00:00

abstract：:Cold agglutinin syndrome (CAS) is usually associated with IgM cold agglutinins with titres exceeding 1000 at 4 degrees C and a thermal amplitude of 30-32 degrees C. Occasionally patients are encountered who although having clinical and laboratory findings compatible with CAS do not have the characteristic serological ...

journal_title：British journal of haematology

authors： Garratty G,Petz LD,Hoops JK

更新日期：1977-04-01 00:00:00

abstract：:B cell receptor (BCR) signalling is an important pathway in diffuse large B cell lymphoma (DLBCL). In response to BCR triggering, normal and malignant B cells secrete the chemokines CCL3 and CCL4 to attract accessory cells to the tissue microenvironment. We measured CCL3 and CCL4 serum concentrations in 102 patients w...

journal_title：British journal of haematology

authors： Takahashi K,Sivina M,Hoellenriegel J,Oki Y,Hagemeister FB,Fayad L,Romaguera JE,Fowler N,Fanale MA,Kwak LW,Samaniego F,Neelapu S,Xiao L,Huang X,Kantarjian H,Keating MJ,Wierda W,Fu K,Chan WC,Vose JM,O'Brien S,Davi

更新日期：2015-12-01 00:00:00

abstract：:Culture medium was harvested after co-incubation of mononuclear cells collected and pooled from the peripheral blood of two different normal donors and was tested for colony-stimulating activity (CSA) in agar culture. With bone marrow from normal donors or peripheral blood from patients with chronic granulocytic leuka...

journal_title：British journal of haematology

authors： Hellmann A,Th'ng KH,Goldman JM

更新日期：1980-06-01 00:00:00

abstract：:Point-of-care testing (POCT) in haematology has continued to grow in popularity and uptake throughout the world. The increasing demand to reduce the turnaround time of test results, coupled with rapid improvements in technology, have led to the development of several devices that are designed for use in different clin...

journal_title：British journal of haematology

authors： Briggs C,Kimber S,Green L

更新日期：2012-09-01 00:00:00

abstract：:Dyskeratosis congenita (DC) is an inherited disorder characterized by skin pigmentation, nail dystrophy and mucosal leucoplakia. In 1995 a Dyskeratosis Congenita Registry was established at the Hammersmith Hospital. In the 46 families recruited, 76/83 patients were male, suggesting that the major form of DC is X-linke...

journal_title：British journal of haematology

authors： Knight S,Vulliamy T,Copplestone A,Gluckman E,Mason P,Dokal I

更新日期：1998-12-01 00:00:00