Elevated urinary levels of thromboxane and prostacyclin metabolities in sickle cell disease reflects activated platelets in the circulation.


:There is evidence for increased factor VII turnover and the associated increased thrombin generation and fibrinolytic activities in sickle cell disease (SCD) that may affect in vivo platelet and endothelial cell reactivity. We studied the release of specific eicosanoids that are indicative of in vivo platelet activation and endothelial cell injury. The circulating and urinary levels of 2,3-dinor thromboxane B2(2,3-dinor-TxB2),TxB2,PGI2 [as 6-keto-PGF1 alpha], and PGE2 were measured in 15HbSS patients, eight HbAA non-haemolytic anaemic individuals and 12 healthy HbAA controls using specific RIAs. The mean urinary 2,3-dinor-TxB2 in the HbSS patients was significantly higher than in both the healthy HbAA and the anaemic controls. 6-keto-PGF1 alpha was undetected in the urines of the healthy HbAA controls, but was measured insignificant amounts in the HbSS and the HbAA anaemic patients. The urinary concentrations of PGE2 and TxB2 in HbSS patients' samples were also significantly higher than those of both control groups (P < 0.05). PGE2 and TxB2 levels were below the detection limit in the plasmas of the HbAA subjects, but were measurable in the HbSS and HbAA anaemic plasmas. The plasma level of 6-keto-PGF1 alpha in the HbSS patients was also significantly higher than in the control groups. The data indicates a persistent inflammatory process in the HbSS patients, and is consistent with the hypothesis that there is platelet and endothelial cell activation in SCD.


Br J Haematol


Kurantsin-Mills J,Ibe BO,Natta CL,Raj JU,Siegel RS,Lessin LS




Has Abstract


1994-07-01 00:00:00












  • Bcl-2 rearrangements with breakpoints in both vcr and mbr in non-Hodgkin's lymphomas and chronic lymphocytic leukaemia.

    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

    pub_type: 杂志文章


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

    更新日期:1996-03-01 00:00:00

  • Thalassaemia-like carriers not linked to the beta-globin gene cluster.

    abstract::This study describes the largest series reported to date, of individuals belonging to unrelated families carrying a beta-thalassaemia-like phenotype in whom the beta-globin gene was found to be structurally intact by sequence analysis. This genetic determinant appears haematologically heterogeneous, displaying either ...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Faà V,Meloni A,Moi L,Ibba G,Travi M,Vitucci A,Cao A,Rosatelli MC

    更新日期:2006-03-01 00:00:00

  • Proposals for the classification of the myelodysplastic syndromes.

    abstract::New diagnostic criteria for the diagnosis of the various myelodysplastic syndromes (MDS) are proposed, and a detailed description is given of the features that may help define MDS. Five MDS are described: (1) refractory anaemia (RA), (2) RA with ring sideroblasts, (3) RA with excess of blasts (RAEB), (4) chronic myelo...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Bennett JM,Catovsky D,Daniel MT,Flandrin G,Galton DA,Gralnick HR,Sultan C

    更新日期:1982-06-01 00:00:00

  • Activation of class I HLA expression by TNF-alpha and gamma-interferon is mediated through protein kinase C-dependent pathway in CML cell lines.

    abstract::The combination of tumour necrosis factor alpha (TNF alpha) and gamma-interferon induced transcription of class I HLA genes in chronic myelogenous leukaemia (CML) cell lines through the formation of a complex between nuclear proteins and the transcriptional enhancers associated with these genes. Although gamma-interfe...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Seong D,Sims S,Johnson E,Lyding J,Lopez A,Garovoy M,Talpaz M,Kantarjian H,Lopez-Berestein G,Reading C

    更新日期:1991-07-01 00:00:00

  • A phase 2 trial of high dose lenalidomide in patients with relapsed/refractory higher-risk myelodysplastic syndromes and acute myeloid leukaemia with trilineage dysplasia.

    abstract::Limited therapies exist for patients with refractory and relapsed (RR) higher-risk myelodysplastic syndromes (HR-MDS) and acute myeloid leukaemia with trilineage dysplasia (AML-TD). High dose (HD) lenalidomide (50 mg) has activity as frontline therapy in elderly AML but there is limited data in the RR setting. This ph...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Zeidan AM,Smith BD,Carraway HE,Gojo I,DeZern A,Gore SD

    更新日期:2017-01-01 00:00:00

  • Integrated DNA copy number and methylation profiling of lymphoid neoplasms using a single array.

    abstract::Changes in DNA copy number (CN) and DNA methylation represent important aberrations for lymphomas and other cancers. Here, for the first time, we show that the Illumina Infinium Methylation (IIM) assay, although not originally designed for CN profiling, is able to estimate CN changes. We compared the IIM CN profiles t...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Kwee I,Rinaldi A,Rancoita P,Rossi D,Capello D,Forconi F,Giuliani N,Piva R,Inghirami G,Gaidano G,Zucca E,Bertoni F

    更新日期:2012-02-01 00:00:00

  • Safety profile of the oral iron chelator deferiprone: a multicentre study.

    abstract::In previous trials, the orally active iron chelator deferiprone (L1) has been associated with sporadic agranulocytosis, milder forms of neutropenia and other side-effects. To determine the incidence of these events, we performed a multicentre prospective study of the chelator. Blood counts were performed weekly, and c...

    journal_title:British journal of haematology

    pub_type: 杂志文章,多中心研究


    authors: Cohen AR,Galanello R,Piga A,Dipalma A,Vullo C,Tricta F

    更新日期:2000-02-01 00:00:00

  • Inactivation of the retinoblastoma gene appears to be very uncommon in myelodysplastic syndromes.

    abstract::Rearrangements of the retinoblastoma (RB) gene have been reported in a few cases of myelodysplastic syndromes (MDS). In addition, low or absent expression of the RB protein is found in 20-30% of cases of acute myeloid leukaemias (AML), particularly in AML with a monocytic component (M4 or M5). We performed Southern bl...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Preudhomme C,Vachee A,Lepelley P,Vanrumbeke M,Zandecki M,Quesnel B,Cosson A,Fenaux P

    更新日期:1994-05-01 00:00:00

  • Acute tumour lysis syndrome: a case in AL amyloidosis.

    abstract::Tumour lysis syndrome (TLS) in plasma cell dyscrasias is extremely rare. TLS has been described in eight cases of multiple myeloma undergoing high-dose therapy with autologous stem cell transplant (ASCT). Recently, clinical trials of intensive chemotherapy followed by autologous or allogeneic stem cell support has bee...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Akasheh MS,Chang CP,Vesole DH

    更新日期:1999-11-01 00:00:00

  • Circumvention of P-glycoprotein-mediated drug resistance in human leukaemic cells by non-immunosuppressive cyclosporin D analogue, SDZ PSC 833.

    abstract::Cyclosporin A (CSA) exhibits greater multidrug resistance (MDR) modulating activity in vitro than other MDR modulators such as verapamil and quinidine. However, the immunosuppressive and nephrotoxic effects of CSA may limit its clinical use. PSC 833, a new cyclosporin D derivative, exerts a higher MDR reversal activit...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Jiang XR,Kelsey SM,Wu YL,Newland AC

    更新日期:1995-06-01 00:00:00

  • CD99 expression is strongly associated with clinical outcome in children with B-cell precursor acute lymphoblastic leukaemia.

    abstract::Our study aimed to determine the expression pattern and clinical relevance of CD99 in paediatric B-cell precursor acute lymphoblastic leukaemia (BCP-ALL). Our findings demonstrate that high expression levels of CD99 are mainly found in high-risk BCP-ALL, e.g. BCR-ABL1 and CRLF2Re/Hi, and that high CD99 mRNA levels are...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Chen D,Camponeschi A,Wu Q,Gerasimcik N,Li H,Shen X,Tan Y,Sjögren H,Nordlund J,Lönnerholm G,Abrahamsson J,Fogelstrand L,Mårtensson IL

    更新日期:2019-02-01 00:00:00

  • Apoptosis in chronic myeloid leukaemia: normal responses by progenitor cells to growth factor deprivation, X-irradiation and glucocorticoids.

    abstract::Inhibition of apoptosis (genetically programmed active cell death) by p210 BCR-ABL expression is a mechanism that might contribute to clonal expansion in chronic myeloid leukaemia (CML). Since cell death following exposure to ionizing radiation and many chemotherapeutic agents can occur by the apoptotic pathway, inhib...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Amos TA,Lewis JL,Grand FH,Gooding RP,Goldman JM,Gordon MY

    更新日期:1995-10-01 00:00:00

  • Red blood cell aggregation and sedimentation: the role of the cell shape.

    abstract::The influence of erythrocyte shape changes on the sedimentation rate was studied in vitro and in vivo. In vitro the highest sedimentation rate was observed with a slight degree of stomatocytosis (morphological index-0.3; i.e. one red cell out of three being a stomatocyte I). With increasing degrees of stomatocytosis t...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Reinhart WH,Singh A,Straub PW

    更新日期:1989-12-01 00:00:00

  • Plasma levels of platelet factor 4 measured by radioimmunoassay.

    abstract::A radioimmunoassay has been developed to measure platelet factor 4 (PF4) in biological fluids both in vitro and in vivo. The assay has been shown to be highly specific for PF4 and has a sensitivity of 0.08 ng/assay tube and 1.6 ng/ml of plasma. The preparation of plasma for the measurement of in vivo levels of PF4 req...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Chesterman CN,McGready JR,Doyle DJ,Morgan FJ

    更新日期:1978-11-01 00:00:00

  • Identification of del(6)(q21q25) as a recurring chromosomal abnormality in putative NK cell lymphoma/leukaemia.

    abstract::The putative natural killer (NK) cell lymphomas/leukaemias are a group of recently characterized haematolymphoid malignancies sharing an immunophenotype of CD3/Leu4- CD3epsilon+ CD56+, and a genotype of germline T-cell receptor genes. They frequently present in extranodal sites and exhibit a highly aggressive clinical...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Wong KF,Chan JK,Kwong YL

    更新日期:1997-09-01 00:00:00

  • Sphingosine-1-phosphate receptor 1 as a prognostic biomarker and therapeutic target for patients with primary testicular diffuse large B-cell lymphoma.

    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

    pub_type: 杂志文章


    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

  • Does sticky blood predict a sticky end? Associations of blood viscosity, haematocrit and fibrinogen with mortality in the West of Scotland.

    abstract::There is increasing evidence that blood viscosity and its major determinants (haematocrit, plasma viscosity and fibrinogen) are associated with an increased risk of incident cardiovascular events; however, their associations with mortality are not established. We therefore studied the associations of these variables w...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Woodward M,Rumley A,Tunstall-Pedoe H,Lowe GD

    更新日期:2003-08-01 00:00:00

  • The role of lipids in the detection of lupus anticoagulant by the dilute Russell Viper venom test: are platelets or reagents containing hexagonal HII phases necessary?

    abstract::Liposomes prepared from rabbit brain extracts (RBE) and individual pure lipids (high phosphatidyl serine content, HIPS) were compared with frozen-thawed platelets (PLTS) in the dilute Russell Viper venom time (dRVVt). While all three preparations demonstrated sensitivity to lupus anticoagulant (LA) the highest detecti...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Stevenson KJ,Seddon JM

    更新日期:1994-03-01 00:00:00

  • Adjusted dose continuous infusion of factor VIII in patients with haemophilia A.

    abstract::Surgical interventions in patients suffering from haemophilia A require infusions of large doses of factor VIII (F VIII) concentrates. These are expensive and may pose a burden on the immune system, which is already compromised in many haemophiliacs. F VIII is usually given as bolus injections, although there are repo...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Martinowitz U,Schulman S,Gitel S,Horozowski H,Heim M,Varon D

    更新日期:1992-12-01 00:00:00

  • OKT-3-based reconditioning regimen for early graft failure in HLA-non-identical stem cell transplants.

    abstract::Primary non-engraftment or early rejection after transplantation of haematopoietic stem cells represent life-threatening complications of allogeneic stem cell transplantation. Management of early graft failure has been problematic, as the risk of fatal infectious complications increases with the time of pancytopenia a...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Schlegel PG,Eyrich M,Bader P,Handgretinger R,Lang P,Niethammer D,Klingebiel T

    更新日期:2000-11-01 00:00:00

  • MOZ/TIF2-induced acute myeloid leukaemia in transgenic fish.

    abstract::The inv(8)(p11q13) chromosomal abnormality, described in acute myeloid leukaemias (AML), fuses the histone acetyl-transferase (HAT) MYST3 (MOZ) gene with another HAT gene, NCOA2 (TIF2). We generated a transgenic zebrafish in which the MYST3/NCOA2 fusion gene was expressed under control of the spi1 promoter. An AML dev...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Zhuravleva J,Paggetti J,Martin L,Hammann A,Solary E,Bastie JN,Delva L

    更新日期:2008-11-01 00:00:00

  • Loss of HLA molecules in B lymphomas is associated with an aggressive clinical course.

    abstract::Major histocompatibility complex class I molecule expression is reduced in some malignant tumours permitting escape from immune surveillance and is therefore associated with a poor prognosis. Seven cases of non-Hodgkin lymphomas out of 300 cases of malignant lymphoproliferative disorders totally lacked expression of c...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Amiot L,Onno M,Lamy T,Dauriac C,Le Prise PY,Fauchet R,Drenou B

    更新日期:1998-03-01 00:00:00

  • Isolation and culture of endothelial cells from human bone marrow.

    abstract::Adhesive interactions between haemopoietic progenitor cells and bone marrow sinusoidal endothelium are potentially important in the homing of these cells back to the extravascular compartment of the marrow to re-establish haemopoiesis following stem cell transplantation. A simple method for the isolation and culture o...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Masek LC,Sweetenham JW

    更新日期:1994-12-01 00:00:00

  • Vincristine-induced apoptosis in vivo in peripheral blood mononuclear cells of children with acute lymphoblastic leukaemia (ALL).

    abstract::We conducted a study to demonstrate vincristine-induced apoptosis in vivo in peripheral blood mononuclear cells of children with newly diagnosed acute lymphoblastic leukaemia (ALL). In five children, apoptosis was detected by terminal deoxynucleotide transferase-mediated dUTP-digoxigenin nick-end labelling (TUNEL) ass...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Groninger E,de Graaf SS,Meeuwsen-de Boer GJ,Sluiter WJ,Poppema S

    更新日期:2000-12-01 00:00:00

  • Mucormycosis in children with haematological malignancies is a salvageable disease: a report from the Israeli Study Group of Childhood Leukemia.

    abstract::Mucormycosis has emerged as an increasingly important cause of morbidity and mortality in immunocompromised patients, but contemporary data in children are lacking. We conducted a nationwide multicentre study to investigate the characteristics of mucormycosis in children with haematological malignancies. The cohort in...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Elitzur S,Arad-Cohen N,Barg A,Litichever N,Bielorai B,Elhasid R,Fischer S,Fruchtman Y,Gilad G,Kapelushnik J,Kharit M,Konen O,Laor R,Levy I,Raviv D,Shachor-Meyouhas Y,Shvartser-Beryozkin Y,Toren A,Yaniv I,Nirel R,I

    更新日期:2020-04-01 00:00:00

  • Molecular pathology of haemoglobin H disease in Sardinians.

    abstract::We investigated the molecular basis for haemoglobin H disease in 50 Sardinian patients by restriction endonuclease analysis. We found that the majority (78% of the cases) are due to gene deletion (- -/- alpha). Among those with a combination of deletion and nondeletion defects (- -/alpha alpha th), the most prevalent ...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Paglietti E,Galanello R,Moi P,Pirastu M,Cao A

    更新日期:1986-07-01 00:00:00

  • Novel alleles at the JK blood group locus explain the absence of the erythrocyte urea transporter in European families.

    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

    pub_type: 杂志文章


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

    更新日期:2002-02-01 00:00:00

  • Clinical use of unfractionated heparin therapy in children: time for change?

    abstract::Paediatric recommendations for unfractionated heparin (UFH) management are extrapolated from adult trials, a practice that may contribute to the inferior UFH-related outcomes in children compared to adults. This is the first study to determine UFH concentration in a population of children and correlated UFH concentrat...

    journal_title:British journal of haematology

    pub_type: 临床试验,杂志文章


    authors: Newall F,Ignjatovic V,Johnston L,Summerhayes R,Lane G,Cranswick N,Monagle P

    更新日期:2010-09-01 00:00:00

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

    abstract::Shwachman-Diamond Syndrome (SDS) is a multi-system genetic disorder with bone marrow failure. SBDS, the gene associated with SDS, has been postulated to play a role in ribosome biogenesis and RNA processing, but its functions are still unknown. To study whether these pathways are interrupted when Sbds protein is lost,...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Rujkijyanont P,Adams SL,Beyene J,Dror Y

    更新日期:2009-06-01 00:00:00

  • Clinico-biological characteristics and treatment of type I monoclonal cryoglobulinaemia: a study of 64 cases.

    abstract::This retrospective analysis was conducted in 64 patients diagnosed with type I cryoglobulinaemia (CG) followed at two French centres. Median follow-up was 6·75 years. CG was IgG in 60% and IgM in 40% of all cases and was asymptomatic in 16 patients (25%). Cold-triggered ischaemic skin manifestations were observed in 3...

    journal_title:British journal of haematology

    pub_type: 杂志文章,多中心研究,随机对照试验


    authors: Harel S,Mohr M,Jahn I,Aucouturier F,Galicier L,Asli B,Malphettes M,Szalat R,Brouet JC,Lipsker D,Fermand JP

    更新日期:2015-03-01 00:00:00