Immune parameter analysis of children with sickle cell disease on hydroxycarbamide or chronic transfusion therapy.


:Sickle cell disease (SCD) is increasingly appreciated as an inflammatory condition associated with alterations in immune phenotype and function. In this cross-sectional study we performed a multiparameter analysis of 18 immune markers in 114 paediatric SCD patients divided by treatment group [those receiving hydroxycrabamide (HC, previously termed hydroxyurea), chronic transfusion (CT), or no disease-modifying therapy] and 29 age-matched African American healthy controls. We found global elevation of most immune cell counts in SCD patients receiving no disease-modifying therapy at steady state. Despite the decrease in percentage of haemoglobin S associated with CT therapy, the abnormal cellular immune phenotype persisted in patients on CT. In contrast, in both univariate and multivariate analysis, treatment with HC was associated with normalization of the vast majority of leucocyte populations. This study provides additional support for HC treatment in SCD, as it appears that HC decreases the abnormally elevated immune cell counts in patients with SCD.


Br J Haematol


Nickel RS,Osunkwo I,Garrett A,Robertson J,Archer DR,Promislow DE,Horan JT,Hendrickson JE,Kean LS




Has Abstract


2015-05-01 00:00:00












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

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    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

  • Aspects of folate metabolism in renal failure.

    abstract::Plasma and urine folate fractions were evaluated after ingestion of radioactive N5-methyl-tetrahydrofolic acid by a normal control (subject I), a patient on maintenance haemodialysis for chronic glomerulonephritis (subject 2), and an anephric patient on haemodialysis (subject 3). In subjects 1 and 2 maximal plasma rad...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Retief FP,Heyns AP,Oosthuizen M,Reenen OR

    更新日期:1977-07-01 00:00:00

  • Deletions of the long arm of chromosome 7 in myeloid disorders: loss of band 7q32 implies worst prognosis.

    abstract::Clinical and cytogenetic data were analysed in 54 patients with acute non-lymphocytic leukaemias (ANLL) or MDS (myelodysplastic syndromes) and deletion of the long arm of chromosome 7 (7q-), in order to determine if there is a commonly deleted region in 7q and to establish possible correlations between karyotypic feat...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Velloso ER,Michaux L,Ferrant A,Hernandez JM,Meeus P,Dierlamm J,Criel A,Louwagie A,Verhoef G,Boogaerts M,Michaux JL,Bosly A,Mecucci C,Van den Berghe H

    更新日期:1996-03-01 00:00:00

  • Translocation t(3;17)(q23;q21): a new translocation in acute lymphoblastic leukaemia.

    abstract::We report on two adult patients with CD10+ positive acute lymphoblastic leukaemia (ALL) who presented with similar clinical and laboratory features and with a new chromosomal translocation: t(3;17)(q23;q21). This translocation may be involved in the formation of a new chimaeric transcription factor. Both patients shar...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Raanani P,Berkowicz M,Rosner E,Rosenthal E,Berman S,Rechavi G,Ben-Bassat I

    更新日期:1996-10-01 00:00:00

  • Asynchronous expression of granulocyte membrane receptors in megaloblastic anaemia.

    abstract::The expression of Fc(IgG) and C3b membrane receptors by granulocytes and their precursors was examined in 23 cases of megaloblastic anaemia which were graded I-III according to morphological severity. Fractionated bone marrow and peripheral blood granulocyte receptors were assessed by rosette formation with optimally ...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Scott CS,Bynoe AG,Roberts BE,Hough D

    更新日期:1982-11-01 00:00:00

  • Unbalanced X-chromosome inactivation in haemopoietic cells from normal women.

    abstract::We studied X-chromosome inactivation patterns in blood cells from normal females in three age groups: neonates (umbilical cord blood), 25-32 years old (young women group) and >75 years old (elderly women). Using PCR, the differential allele methylation status was evaluated on active and inactive X chromosomes at the h...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Tonon L,Bergamaschi G,Dellavecchia C,Rosti V,Lucotti C,Malabarba L,Novella A,Vercesi E,Frassoni F,Cazzola M

    更新日期:1998-09-01 00:00:00

  • The British Society for Haematology: 'What have the Romans ever done for us?'.

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    journal_title:British journal of haematology

    pub_type: 杂志文章,评审


    authors: Baglin T,Carrington P,Jackson G,Board of Trustees of the BSH.

    更新日期:2015-05-01 00:00:00

  • High-dose therapy improves the bone remodelling compartment canopy coverage and bone formation in multiple myeloma.

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    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Hinge M,Delaisse JM,Plesner T,Clasen-Linde E,Salomo M,Andersen TL

    更新日期:2015-11-01 00:00:00

  • Multiple in silico tools predict phenotypic manifestations in congenital thrombotic thrombocytopenic purpura.

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    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Hing ZA,Schiller T,Wu A,Hamasaki-Katagiri N,Struble EB,Russek-Cohen E,Kimchi-Sarfaty C

    更新日期:2013-03-01 00:00:00

  • Protein C and protein S levels in two patients with acquired purpura fulminans.

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    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Madden RM,Gill JC,Marlar RA

    更新日期:1990-05-01 00:00:00

  • Less than half of patients aged 65 years or under with myeloma proceed to transplantation: results of a two region population-based survey.

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    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Morris TC,Velangi M,Jackson G,Marks DI,Ranaghan L,Northern Ireland Regional Haematology Group.,Northern Regional Haematologists Group.,Clinical Trials Committee of The British Scoiety for Blood and Marrow Transplantation.

    更新日期:2005-02-01 00:00:00

  • Factors affecting cardiolipin antibody assays: modification with polyethylene glycol compound.

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    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Kilpatrick DC

    更新日期:1998-01-01 00:00:00

  • Clonal disease of natural killer large granular lymphocytes in Taiwan.

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    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Chou WC,Chiang IP,Tang JL,Su IJ,Huang SY,Chen YC,Liu MC,Lee FY,Wang CH,Shen MC,Chuang SM,Tien HF

    更新日期:1998-12-01 00:00:00

  • Is the beta thalassaemia trait of clinical importance?

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    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Premawardhena A,Arambepola M,Katugaha N,Weatherall DJ

    更新日期:2008-05-01 00:00:00

  • Polymorphism in CD33 and CD34 genes: a source of minor histocompatibility antigens on haemopoietic progenitor cells?

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    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Raptis A,Clave E,Mavroudis D,Molldrem J,Van Rhee F,Barrett AJ

    更新日期:1998-09-01 00:00:00

  • The clinical impact of staging bone marrow examination on treatment decisions and prognostic assessment of lymphoma patients.

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    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Painter D,Smith A,de Tute R,Crouch S,Roman E,Jack A

    更新日期:2015-07-01 00:00:00

  • Leucocyte alkaline phosphatase identifies terminally differentiated normal neutrophils and its lack in chronic myelogenous leukaemia is not dependent on p210 tyrosine kinase activity.

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    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Dotti G,Garattini E,Borleri G,Masuhara K,Spinelli O,Barbui T,Rambaldi A

    更新日期:1999-04-01 00:00:00

  • Hypercalcaemia complicating acute myelogenous leukaemia: a syndrome of multiple aetiologies.

    abstract::Hypercalcaemia complicating acute myelogenous leukaemia is a rare but well-recognized phenomenon. In most cases the pathogenetic mechanism causing the hypercalcaemia remains poorly understood. We recently studied in detail two patients with acute myelogenous leukaemia who developed hypercalcaemia during the course of ...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Gewirtz AM,Stewart AF,Vignery A,Hoffman R

    更新日期:1983-05-01 00:00:00

  • Lessons in platelet production from inherited thrombocytopenias.

    abstract::Our knowledge of the cellular and molecular mechanisms of platelet production has greatly expanded in recent years due to the opportunity to culture in vitro megakaryocytes and to create transgenic animals with specific genetic defects that interfere with platelet biogenesis. However, in vitro models do not reproduce ...

    journal_title:British journal of haematology

    pub_type: 杂志文章,评审


    authors: Pecci A,Balduini CL

    更新日期:2014-04-01 00:00:00

  • Real world data as a key element in precision medicine for lymphoid malignancies: potentials and pitfalls.

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    journal_title:British journal of haematology

    pub_type: 杂志文章,评审


    authors: El-Galaly TC,Cheah CY,Villa D

    更新日期:2019-08-01 00:00:00

  • Antithrombins Southport (Leu 99 to Val) and Vienna (Gln 118 to Pro): two novel antithrombin variants with abnormal heparin binding.

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    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Chowdhury V,Mille B,Olds RJ,Lane DA,Watton J,Barrowcliffe TW,Pabinger I,Woodcock BE,Thein SL

    更新日期:1995-03-01 00:00:00

  • Reduced intensity allogeneic stem cell transplantation for younger patients with myelofibrosis.

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    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Mannina D,Zabelina T,Wolschke C,Heinzelmann M,Triviai I,Christopeit M,Badbaran A,Bonmann S,von Pein UM,Janson D,Ayuk F,Kröger N

    更新日期:2019-08-01 00:00:00

  • Short-term study of chimaerism after bone marrow transplantation for severe aplastic anaemia.

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    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Socié G,Landman J,Gluckman E,Devergie A,Raynal B,Esperou-Bourdeau H,Brison O

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  • Flow cytometry detection of serotonin content and release in resting and activated platelets.

    abstract::Early detection of platelet activation is important for the diagnosis and follow-up of several pathological conditions that primarily or secondarily involve platelets in their pathogenesis. The golden standard assay to detect thrombocyte activation is represented by the release of serotonin, classically performed by d...

    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Gobbi G,Mirandola P,Tazzari PL,Ricci F,Caimi L,Cacchioli A,Papa S,Conte R,Vitale M

    更新日期:2003-06-01 00:00:00

  • Primary thrombocythaemia associated with systemic mastocytosis: a report of five cases.

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    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Le Tourneau A,Gaulard P,D'Agay MF,Vainchencker W,Cadiou M,Devidas A,Haioun C,Clauvel JP,Audouin J,Diebold J

    更新日期:1991-09-01 00:00:00

  • Differential binding of IgG anti-D and IgG autoantibodies to reticulocytes and red blood cells.

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    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Gray LS,Kleeman JE,Masouredis SP

    更新日期:1983-10-01 00:00:00

  • Serum "uracil+uridine" levels in pernicious anaemia.

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    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Parry TE,Blackmore JA

    更新日期:1976-12-01 00:00:00

  • Tandem transplants with different high-dose regimens improve the complete remission rates in multiple myeloma. Results of a Grupo Español de Síndromes Linfoproliferativos/Trasplante Autólogo de Médula Osea phase II trial.

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    journal_title:British journal of haematology

    pub_type: 临床试验,杂志文章,多中心研究


    authors: Lahuerta JJ,Grande C,Martínez-Lopez J,De La Serna J,Toscano R,Ortiz MC,Larregla S,Conde E,Insunza A,Gonzalez-San Miguel JD,Bargay J,Cabrera R,García-Ruiz JC,Albó C,García-Alonso L,Solano F,Vivancos P,León A,San Miguel

    更新日期:2003-01-01 00:00:00

  • Interferon alpha and zidovudine therapy in adult T-cell leukaemia lymphoma: response and outcome in 15 patients.

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    journal_title:British journal of haematology

    pub_type: 杂志文章


    authors: Matutes E,Taylor GP,Cavenagh J,Pagliuca A,Bareford D,Domingo A,Hamblin M,Kelsey S,Mir N,Reilly JT

    更新日期:2001-06-01 00:00:00

  • Roles for both cyclic GMP and cyclic AMP in the inhibition of collagen-induced platelet aggregation by nitroprusside.

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    journal_title:British journal of haematology

    pub_type: 杂志文章


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

    更新日期:2002-06-01 00:00:00