Severe prekallikrein deficiency associated with homozygosity for an Arg94Stop nonsense mutation.

abstract：:Murine monoclonal antibodies (MoAbs) were produced against the blood group KEL1 glycoprotein (93 kD component) immunopurified from human erythrocytes. One monoclonal antibody, 5A11 (IgGa, kappa), detects by immunoblotting a 93 and 184 kD component from KEL: 1,-2 or KEL: -1,2 red cell membrane preparations, separated b...

journal_title：British journal of haematology

authors： Jaber A,Loirat MJ,Willem C,Bloy C,Cartron JP,Blanchard D

更新日期：1991-10-01 00:00:00

abstract：:The alpha+ thalassaemias are the most common single gene disorders of humans, yet little is known about their haematological characteristics in childhood. Blood samples have been collected randomly from more than 2000 individuals in village communities in Vanuatu in the South West Pacific and analysed for alpha thalas...

journal_title：British journal of haematology

authors： Williams TN,Maitland K,Ganczakowski M,Peto TE,Clegg JB,Weatherall DJ,Bowden DK

更新日期：1996-11-01 00:00:00

abstract：:Polymerase chain reaction protocols were designed specifically to amplify regions of the alpha globin complex that contain the nine common polymorphic haplotyping sites. These reactions provided a quicker and more sensitive approach to determining alpha globin haplotypes than Southern blotting methods. ...

journal_title：British journal of haematology

authors： Miles KL,Norwich JT,Martinson JJ,Clegg JB

更新日期：2001-06-01 00:00:00

abstract：:We report a high incidence of subacute, chronic and sometimes occult intracranial subdural haematoma (SDH) occurring during intensive chemotherapy for acute myeloid leukaemia (AML) with a monoblastic component. Between March 1990 and January 1993, 86 AML patients from our institution were randomized in the multicentri...

journal_title：British journal of haematology

authors： Jourdan E,Dombret H,Glaisner S,Micléa JM,Castaigne S,Degos L

更新日期：1995-03-01 00:00:00

abstract：:Dendritic cell (DC) differentiation was investigated in samples from two acute promyelocytic leukaemia (APL) patients with classic translocation t(15;17)(q22;q21). After 18 d of culture in the presence of granulocyte-macrophage colony-stimulating factor, interleukin 4 and tumour necrosis factor alpha, 10-15% of pathol...

journal_title：British journal of haematology

authors： Rigolin GM,Della Porta M,Bigoni R,Tieghi A,Cuneo A,Castoldi G

更新日期：2001-09-01 00:00:00

abstract：:The bcl-2 gene is rearranged in most cases of follicular lymphoma and the breakpoint clusters into two specific regions: mbr and mcr. Rearrangements to immunoglobulin heavy chain genes (IgH) result in a deregulation of the gene and increased transcription of mRNA for the bcl-2 protein. In chronic lymphocytic leukaemia...

journal_title：British journal of haematology

authors： Merup M,Spasokoukotskaja T,Einhorn S,Smith CI,Gahrton G,Juliusson G

更新日期：1996-03-01 00:00:00

abstract：:The economic impact of using prophylactic clodronate as an adjunct to chemotherapy in the management of multiple myeloma for the first 4 years following diagnosis was established from the perspective of the National Health Service (NHS). A state-transition model of the course of multiple myeloma was constructed using ...

journal_title：British journal of haematology

authors： Bruce NJ,McCloskey EV,Kanis JA,Guest JF

更新日期：1999-02-01 00:00:00

abstract：:We assessed the role of human CD52 antibody (Campath-1H) in six patients with chronic lymphocytic leukaemia (CLL) treated to maximal response with purine analogues (fludarabine/deoxycoformycin) in whom persistent leukaemic infiltration of blood and bone marrow had precluded autologous stem cell transplantation. Five p...

journal_title：British journal of haematology

authors： Dyer MJ,Kelsey SM,Mackay HJ,Emmett E,Thornton P,Hale G,Waldmann H,Newland AC,Catovsky D

更新日期：1997-06-01 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：:Serum erythropoietin (Epo) was measured in 23 patients before, during and after intensive cytostatic treatment courses for acute leukaemia or before bone marrow transplantation. A marked increase was seen in all patients, starting 1 or 2 d after initiation of treatment. A peak was reached after about 7 d, at levels as...

journal_title：British journal of haematology

authors： Birgegård G,Wide L,Simonsson B

更新日期：1989-07-01 00:00:00

abstract：:We describe five adult patients with sickle cell anaemia (SS) who developed clinical, radiological and histological evidence of splenic regrowth while receiving regular blood transfusions. Five patients, all homozygous SS, range 23-34 years, were commenced on hypertransfusion therapy. Three patients were transfused be...

journal_title：British journal of haematology

authors： Campbell PJ,Olatunji PO,Ryan KE,Davies SC

更新日期：1997-01-01 00:00:00

abstract：:A case of immune neutropenia following unrelated stem cell transplantation for chronic myeloid leukaemia is described. The neutropenia developed following herpes zoster viral infection and was associated with antibodies to the human neutrophil antigen (HNA)-2a (formerly known as NB1). The neutropenia was prolonged, pr...

journal_title：British journal of haematology

authors： Pocock CF,Lucas GF,Giles C,Vassiliou G,Cwynarski K,Rezvani K,Apperley JF,Goldman JM

更新日期：2001-05-01 00:00:00

abstract：:Children with Down syndrome (DS) have a greater risk for developing both acute lymphoblastic leukaemia (ALL) and significant adverse effects of chemotherapy. We investigated their outcome with, and tolerance of, treatment protocols for relapsed ALL optimized in the paediatric population without DS. Probability of surv...

journal_title：British journal of haematology

authors： Meyr F,Escherich G,Mann G,Klingebiel T,Kulozik A,Rossig C,Schrappe M,Henze G,von Stackelberg A,Hitzler J

更新日期：2013-07-01 00:00:00

abstract：:Acute promyelocytic leukaemia (APL) is a rare subtype of acute myeloid leukaemia. The outcome of paediatric APL has improved substantially over the past 20 years; cure rates above 80% are expected when all-trans retinoic acid (ATRA) is given with anthracycline-based regimens. The presenting features of paediatric APL ...

journal_title：British journal of haematology

authors： Abla O,Ribeiro RC

更新日期：2014-01-01 00:00:00

abstract：:In studies on human platelets, nitroprusside (NP) alone at 1-10 micromol/l increased platelet cyclic AMP (cAMP) by 40-70%, whereas increases in cyclic GMP (cGMP) were much larger in percentage though not in concentration terms. Collagen enhanced these increases in cAMP up to fourfold, without affecting cGMP. This effe...

journal_title：British journal of haematology

authors： Jang EK,Azzam JE,Dickinson NT,Davidson MM,Haslam RJ

更新日期：2002-06-01 00:00:00

abstract：:Neutrophils are amongst the first immune cells to arrive at sites of infection and play an important role as the host's first line of defence against invading pathogens. Defects of neutrophil number or function are usually recognized clinically by recurrent infections that often are life-threatening. Over the last few...

journal_title：British journal of haematology

authors： Bouma G,Ancliff PJ,Thrasher AJ,Burns SO

更新日期：2010-11-01 00:00:00

abstract：:Deregulated Hedgehog (Hh) signalling activity may be associated with a broad range of cancer types and hence has become an attractive target for therapeutic intervention. Although initial haematological interest focused on the therapeutic targeting of this pathway in chronic myeloid leukaemia), small molecule inhibito...

journal_title：British journal of haematology

authors： Khan AA,Harrison CN,McLornan DP

更新日期：2015-08-01 00:00:00

abstract：:An association between chronic hepatitis C virus (HCV) infection and clonal proliferation of B cells, including B cell lymphoma, has recently been demonstrated. However, the mechanism of malignant transformation is still unknown. It has been shown that B cells from patients with type II mixed cryoglobulinaemia (MC), s...

journal_title：British journal of haematology

authors： Zuckerman E,Zuckerman T,Sahar D,Streichman S,Attias D,Sabo E,Yeshurun D,Rowe J

更新日期：2001-02-01 00:00:00

abstract：:Hydroxyurea (HU), an inhibitor of DNA synthesis, can also induce haemoglobinization in certain erythroid cell lines. In this study, we report that intracellular peroxides levels were increased in HU-treated murine erythroleukaemia (MEL) cells and that l-acetyl-N-cysteine (LNAC), a potent reducing reagent, had a signif...

journal_title：British journal of haematology

authors： Nagai T,Tarumoto T,Miyoshi T,Ohmine K,Muroi K,Komatsu N,Sassa S,Ozawa K

更新日期：2003-05-01 00:00:00

abstract：:Early T-cell precursor acute lymphoblastic leukaemia (ETP-ALL) is a recently identified subtype of T-ALL with distinctive gene expression and cell marker profiles, poor response to chemotherapy and a very high risk of relapse. We determined the reliability of restricted panel of cell markers to identify EPT-ALL using ...

journal_title：British journal of haematology

authors： Inukai T,Kiyokawa N,Campana D,Coustan-Smith E,Kikuchi A,Kobayashi M,Takahashi H,Koh K,Manabe A,Kumagai M,Ikuta K,Hayashi Y,Tsuchida M,Sugita K,Ohara A

更新日期：2012-02-01 00:00:00

abstract：:Activating mutations of NOTCH1 are a common occurrence in T-cell acute lymphoblastic leukaemia (T-ALL), but its impact on T-ALL treatment is still controversial. In this study, the incidence, clinical features, and prognosis of 92 Chinese children with T-ALL treated using the Beijing Children's Hospital-2003 and Chine...

journal_title：British journal of haematology

authors： Gao C,Liu SG,Zhang RD,Li WJ,Zhao XX,Cui L,Wu MY,Zheng HY,Li ZG

更新日期：2014-07-01 00:00:00

abstract：:Abnormal adhesive interaction between bone marrow stroma and progenitors, one of the causes of unregulated proliferation in chronic myelocytic leukaemia (CML), may be caused by some alterations in adhesion molecules on CML progenitors. We investigated the expression of adhesion molecules (CD44, VLA-5, VLA-4, LFA-1, IC...

journal_title：British journal of haematology

authors： Kawaishi K,Kimura A,Katoh O,Sasaki A,Oguma N,Ihara A,Satow Y

更新日期：1996-05-01 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：: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：:We report a case of positive Philadelphia chromosome adult acute lymphoblastic leukaemia with a novel unbalanced translocation t(17;19), leading to trisomy of 17q21-qter. The patient did not obtain complete haematological response and died a few months after diagnosis. The significance of the 17q21-qter trisomy, resul...

journal_title：British journal of haematology

authors： Specchia G,Albano F,Anelli L,Storlazzi CT,Cimino G,Liso A,Zagaria A,Liso V,Rocchi M

更新日期：2002-11-01 00:00:00

abstract：:Sphingosine-1-phosphate (S1P) is a potent lipid mediator that is produced during the metabolism of sphingolipid by sphingosine kinase. S1P has been implicated in the migration and trafficking of lymphocytes and several lymphoid malignancies through S1P receptors. Moreover, the overexpression of sphingosine-1-phosphate...

journal_title：British journal of haematology

authors： Koresawa R,Yamazaki K,Oka D,Fujiwara H,Nishimura H,Akiyama T,Hamasaki S,Wada H,Sugihara T,Sadahira Y

更新日期：2016-07-01 00:00:00

abstract：:Platelet function was investigated in three patients with the Lesch-Nyhan syndrome. Platelet count, morphology and size distribution was normal in all patients. Platelet turnover was normal. Electron microscopy did not reveal any ultrastructural abnormality. Template bleeding times were normal and prolonged after aspi...

journal_title：British journal of haematology

authors： Rivard GE,Izadi P,Lazerson J,McLaren JD,Parker C,Fish CH

更新日期：1975-10-01 00:00:00

abstract：:Diamond Blackfan Anaemia (DBA) is a rare congenital pure red cell aplasia that may be associated with facio-skeletal developmental defects. The disease is caused by mutations in one of at least ten ribosomal proteins, which results in haploinsufficiency and an imbalance between the synthesis of rRNA and ribosomal prot...

journal_title：British journal of haematology

authors： Horos R,von Lindern M

更新日期：2012-12-01 00:00:00

abstract：:Fetal haemoglobin levels have been measured prospectively in 135 autopsy cases of sudden, unexpected infant deaths (31 pre-term, 104 full term) using standard laboratory methods. These results have been compared with Hb F values from a normal control group of 570 living infants (145 pre-term, 425 full-term) with a pos...

journal_title：British journal of haematology

authors： Fagan DG,Walker A

更新日期：1992-10-01 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