Osteitis fibrosa cystica generalizata with adult T-cell leukaemia: a case report.

abstract：:Defective marrow stroma or microenvironment have been proposed as one of several mechanisms to account for bone marrow failure in aplastic anaemia (AA). Stem cell factor (SCF), the ligand for the c-kit receptor, is produced mainly by marrow stromal cells and seems to reflect the haemopoietic function of bone marrow st...

journal_title：British journal of haematology

authors： Kojima S,Matsuyama T,Kodera Y

更新日期：1997-11-01 00:00:00

abstract：:We report outcomes after myeloablative haematopoietic cell transplantation (HCT) from human leucocyte antigen (HLA)-matched sibling donors in 67 patients with sickle cell disease transplanted between 1989 and 2002. The most common indications for transplantation were stroke and recurrent vaso-occlusive crisis in 38% a...

journal_title：British journal of haematology

authors： Panepinto JA,Walters MC,Carreras J,Marsh J,Bredeson CN,Gale RP,Hale GA,Horan J,Hows JM,Klein JP,Pasquini R,Roberts I,Sullivan K,Eapen M,Ferster A,Non-Malignant Marrow Disorders Working Committee, Center for International Bl

更新日期：2007-06-01 00:00:00

abstract：:The specific sodium pump antagonist ouabain was used to study the effect of sodium pump inhibition on granulopoietic colony formation by normal human bone marrow cells cultured in soft agar for 7 d. Suppression of colony formation was dose-dependent and occurred at a low and reproducible concentration. The use of an e...

journal_title：British journal of haematology

authors： Steed AJ,Boardman KM,Delamore IW

更新日期：1982-07-01 00:00:00

abstract：:A 14-month-old child who had a haemolytic episode when he was 5 years old, and with psychomotor retardation, was found to have decreased red cell hexokinase activity. The mutant enzyme was characterized by an increased affinity for glucose associated with an increased inhibition constant for glucose-1,6-diphosphate. A...

journal_title：British journal of haematology

authors： Magnani M,Stocchi V,Canestrari F,Dachà M,Balestri P,Farnetani MA,Giorgi D,Fois A,Fornaini G

更新日期：1985-09-01 00:00:00

abstract：:In four unrelated patients with chronic haemolysis and markedly reduced red blood cell (RBC) glutathione (49.5%, 12.6%, 11.5% and 15% of the normal concentration respectively), a severe glutathione synthetase (GSH-S, EC 6.3.2.3) deficiency was found. One case exhibited a neonatal haemolytic anaemia associated with oxo...

journal_title：British journal of haematology

authors： Corrons JL,Alvarez R,Pujades A,Zarza R,Oliva E,Lasheras G,Callis M,Ribes A,Gelbart T,Beutler E

更新日期：2001-02-01 00:00:00

abstract：:Systemic inflammation activates the tissue factor/factor VIIa complex (TF/FVIIa), leading to a procoagulant state, which may be enhanced by impairment of physiological anticoagulant pathways, such as the protein C system. Besides impaired protein C activation, resistance to activated protein C (APC) may occur. We stud...

journal_title：British journal of haematology

authors： de Pont AC,Bakhtiari K,Hutten BA,de Jonge E,Vlasuk GP,Rote WE,Levi M,Büller HR,Meijers JC

更新日期：2006-07-01 00:00:00

abstract：:To study the pathogenesis of multicentric Castleman's disease (MCD), IL-6 producing cells and immune function were investigated in four MCD patients. The expression of IL-6 mRNA in one MCD lymph node was analysed by in situ hybridization. IL-6 mRNA expressing cells were scattered in the interfollicular areas and did n...

journal_title：British journal of haematology

authors： Ishiyama T,Nakamura S,Akimoto Y,Koike M,Tomoyasu S,Tsuruoka N,Murata Y,Sato T,Wakabayashi Y,Chiba S

更新日期：1994-03-01 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：:The dependence of survival time on a set of prognostic factors was explored by means of Cox's regression model in 137 cases of myelofibrosis with myeloid metaplasia (MMM). The following parameters recorded at diagnosis proved to be important independent indicators of a poor prognosis: a higher value for age, a lower v...

journal_title：British journal of haematology

authors： Barosi G,Berzuini C,Liberato LN,Costa A,Polino G,Ascari E

更新日期：1988-12-01 00:00:00

abstract：:Platelet associated C3c and C3d (PAC3c and PAC3d) were quantitated by enzyme linked assay in 105 patients with idiopathic autoimmune thrombocytopenia (AITP) in whom elevated platelet associated immunoglobulins (IgG and IgM) had previously been documented. Increased levels of complement components were demonstrated in ...

journal_title：British journal of haematology

authors： Hegde UM,Bowes A,Roter BL

更新日期：1985-05-01 00:00:00

abstract：:Long term outcomes and mutations in patients with mantle cell lymphoma (MCL) who discontinued ibrutinib have not been described. Using deep targeted next generation sequencing, we performed somatic mutation profiling from 15 MCL patients (including 5 patients with paired samples; before and after progression on ibruti...

journal_title：British journal of haematology

authors： Jain P,Kanagal-Shamanna R,Zhang S,Ahmed M,Ghorab A,Zhang L,Ok CY,Li S,Hagemeister F,Zeng D,Gong T,Chen W,Badillo M,Nomie K,Fayad L,Medeiros LJ,Neelapu S,Fowler N,Romaguera J,Champlin R,Wang L,Wang ML

更新日期：2018-11-01 00:00:00

abstract：:The reasons why some patients with multiple myeloma (MM) do not develop severe bone loss, or even develop sclerotic bone lesions, remain unclear. In order to answer this question at the cellular and tissue level, we evaluated the histological bone condition of 10 patients with MM who never developed lytic bone lesions...

journal_title：British journal of haematology

authors： Bataille R,Chappard D,Marcelli C,Rossi JF,Dessauw P,Baldet P,Sany J,Alexandre C

更新日期：1990-12-01 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：:The efficiency of stored platelet transfusion was evaluated in terms of clinical status in 141 thrombocytopenic patients. In a paired prospective study in which fresh platelets were used as controls, clinical efficiency was assessed on the basis of the ability to increase platelet count (recovery) and the time to the ...

journal_title：British journal of haematology

authors： Norol F,Kuentz M,Cordonnier C,Beaujean F,Haioun C,Vernant JP,Duédari N

更新日期：1994-01-01 00:00:00

abstract：:Many adult T-cell leukaemia/lymphoma (ATLL) patients who respond to induction treatment, then relapse. Knowing the clonality pattern of residual tumourous clones during treatment could help understand disease evolution and aid therapeutic decisions. We developed a sensitive and semi-quantitative molecular analysis of ...

journal_title：British journal of haematology

authors： Leclercq I,Mortreux F,Morschhauser F,Duthilleul P,Desgranges C,Gessain A,Cavrois M,Vernant JP,Hermine O,Wattel E

更新日期：1999-06-01 00:00:00

abstract：:We report a case of de novo acute myelomonocytic leukaemia with the t(2;11)(q31;p15) translocation in a Japanese female infant. The NUP98-HOXD13 fusion transcript generated by the translocation was detected in the patient's bone marrow cells by reverse transcription-polymerase chain reaction (RT-PCR). Additionally, ec...

journal_title：British journal of haematology

authors： Shimada H,Arai Y,Sekiguchi S,Ishii T,Tanitsu S,Sasaki M

更新日期：2000-07-01 00:00:00

abstract：:A novel colony stimulating factor-1 (CSF-1) binding factor present in the serum from a patient in remission from lymphoma is described. Radioimmunoassay (RIA) repeatedly failed to detect circulating levels of CSF-1 in the peripheral blood system of this patient. Molecular analysis showed a normal CSF-1 gene structure ...

journal_title：British journal of haematology

authors： Baker AH,Cachia PG,Tennant GB,Whittaker JA,White D,Stanley ER,Burnett AK,Padua RA

更新日期：1995-01-01 00:00:00

abstract：:Although expressed in several haematological lineages and involved in multiple different signalling pathways, Bruton tyrosine kinase (BTK) plays an indispensible role in B cells in signalling from the B cell receptor (BCR) for antigen. Many B cell malignancies remain dependent on constitutive BCR signalling, making BT...

journal_title：British journal of haematology

authors： Hutchinson CV,Dyer MJ

更新日期：2014-07-01 00:00:00

abstract：:Juvenile haemochromatosis or haemochromatosis type 2 is a rare autosomal recessive disorder which causes iron overload at a young age, affects both sexes equally and is characterized by a prevalence of hypogonadism and cardiopathy. Patients with haemochromatosis type 2 have been reported in different ethnic groups. Li...

journal_title：British journal of haematology

authors： De Gobbi M,Roetto A,Piperno A,Mariani R,Alberti F,Papanikolaou G,Politou M,Lockitch G,Girelli D,Fargion S,Cox TM,Gasparini P,Cazzola M,Camaschella C

更新日期：2002-06-01 00:00:00

abstract：:In 44 of 56 patients with malignant paraproteinaemia the relationship of serum viscosity, plasma volume (PV), estimated blood volume (BV) and clinical findings was investigated and in the remaining 12 patients one or more of these parameters was studied. There was a correlation between increased PV and serum viscosity...

journal_title：British journal of haematology

authors： Russell JA,Powles RL

更新日期：1978-06-01 00:00:00

abstract：:We have studied the surface expression of the Toll-like receptor family member CD 180 on cells from 78 patients with B-chronic lymphocytic leukaemia (B-CLL). B-CLL cells had variable levels of CD 180 expression, but this was always less than that expressed by normal blood B cells and was stable for 24 months. Signific...

journal_title：British journal of haematology

authors： Porakishvili N,Kulikova N,Jewell AP,Youinou PY,Yong K,Nathwani A,Heelan B,Duke V,Hamblin TJ,Wallace P,Ely P,Clark EA,Lydyard PM

更新日期：2005-11-01 00:00:00

abstract：:During the past years, several authors have used labelled leucocytes to detect inflammatory foci. However, before routine use in man. It is necessary to control the viability of labelled cells. Five leucocyte labelling techniques (111In-oxine, 111In-oxine without extraction, 99mTc oxine, pyrophosphate 99mTc, 51Cr) wer...

journal_title：British journal of haematology

authors： Colas-Linhart N,Barbu M,Gougerot MA,Bok B

更新日期：1983-01-01 00:00:00

abstract：:In a uniform series of 170 untreated myeloma patients (MM) we investigated the distribution of T cell subsets in peripheral blood (PB) and their relationship with the most relevant disease characteristics, including survival. CD4 cells were significantly decreased both in percentage and absolute numbers (P less than 0...

journal_title：British journal of haematology

authors： San Miguel JF,González M,Gascón A,Moro MJ,Hernández JM,Ortega F,Jiménez R,Guerras L,Romero M,Casanova F

更新日期：1992-03-01 00:00:00

abstract：:Excessive bleeding represents a major complication of surgical interventions and its control is especially relevant in patients with Congenital Bleeding Disorders (CBD). In factor VII (FVII) deficiency, scanty data on surgery is available to guide treatment strategies. The STER (Seven Treatment Evaluation Registry) is...

journal_title：British journal of haematology

authors： Mariani G,Dolce A,Batorova A,Auerswald G,Schved JF,Siragusa S,Napolitano M,Knudsen JB,Ingerslev J,STER and the International Factor VII Deficiency Study Groups.

更新日期：2011-02-01 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 relationship between the three measurable components of the factor VIII complex, procoagulant activity (VIII:C), Ristocetin cofactor (VIIIR:WF) and factor VIII related antigen (VIIR:AG), has been investigated using a solid phase immunoadsorption system in which homologous antibodies specific for VIII:C are insolu...

journal_title：British journal of haematology

authors： Koutts J,Lavergne JM,Meyer D

更新日期：1977-11-01 00:00:00

abstract：:To identify a commonly deleted region of 13q14 in idiopathic myelofibrosis (IMF), we used fluorescence in situ hybridization analysis to test for deletion of the RB1 and BRCA2 genes, and the microsatellite loci D13S319 and D13S25, in a series of 25 patients. A further two patients with myelofibrosis secondary to polyc...

journal_title：British journal of haematology

authors： Sinclair EJ,Forrest EC,Reilly JT,Watmore AE,Potter AM

更新日期：2001-05-01 00:00:00

abstract：:Risk factors for acute graft-versus-host disease (GvHD) remain controversial. We performed uni- and multivariate statistical analyses on a series of 37 patients receiving a non-depleted allogeneic bone marrow transplant from an HLA-identical sibling donor for a haematological malignancy, in order to identify risk fact...

journal_title：British journal of haematology

authors： Bagot M,Mary JY,Heslan M,Kuentz M,Cordonnier C,Vernant JP,Dubertret L,Levy JP

更新日期：1988-12-01 00:00:00

abstract：:While proteasome inhibition is a validated therapeutic approach for multiple myeloma (MM), inhibition of individual constitutive proteasome (c20S) and immunoproteasome (i20S) subunits has not been fully explored owing to a lack of effective tools. We utilized the novel proteasome constitutive/immunoproteasome subunit ...

journal_title：British journal of haematology

authors： Lee SJ,Levitsky K,Parlati F,Bennett MK,Arastu-Kapur S,Kellerman L,Woo TF,Wong AF,Papadopoulos KP,Niesvizky R,Badros AZ,Vij R,Jagannath S,Siegel D,Wang M,Ahmann GJ,Kirk CJ

更新日期：2016-06-01 00:00:00

abstract：:A case of Evans' syndrome with IgM deficiency and lymphopenia was studied before and after splenectomy. The lymphopenia was as a result of profound reduction of CD4 and CD8 cells. Study of cytokine secretion before splenectomy revealed a spontaneous Th1- and Th2-type cytokine production, and complete suppression of tr...

journal_title：British journal of haematology

authors： Karakantza M,Mouzaki A,Theodoropoulou M,Bussel JB,Maniatis A

更新日期：2000-09-01 00:00:00