Red cell membrane disorders.


:Significant advances have been made in our understanding of the structural basis for altered cell function in various inherited red cell membrane disorders with reduced red cell survival and resulting hemolytic anemia. The current review summarizes these advances as they relate to defining the molecular and structural basis for disorders involving altered membrane structural organization (hereditary spherocytosis [HS] and hereditary elliptocytosis [HE]) and altered membrane transport function (hereditary overhydrated stomatocytosis and hereditary xerocytosis). Mutations in genes encoding membrane proteins that account for these distinct red cell phenotypes have been identified. These molecular insights have led to improved understanding of the structural basis for altered membrane function in these disorders. Weakening of vertical linkage between the lipid bilayer and spectrin-based membrane skeleton leads to membrane loss in HS. In contrast, weakening of lateral linkages among different skeletal proteins leads to membrane fragmentation and decreased surface area in HE. The degrees of membrane loss and resultant increases in cell sphericity determine the severity of anemia in these two disorders. Splenectomy leads to amelioration of anemia by increasing the circulatory red cell life span of spherocytic red cells that are normally sequestered by the spleen. Disordered membrane cation permeability and resultant increase or decrease in red cell volume account for altered cellular deformability of hereditary overhydrated stomatocytosis and hereditary xerocytosis, respectively. Importantly, splenectomy is not beneficial in these two membrane transport disorders and in fact contraindicated due to severe postsplenectomy thrombotic complications.


Int J Lab Hematol


Narla J,Mohandas N




Has Abstract


2017-05-01 00:00:00








39 Suppl 1


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    pub_type: 杂志文章


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    pub_type: 杂志文章,评审


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    pub_type: 杂志文章


    authors: Zhong Y,Feng J,Chen B,Cheng L,Li Y,Qian J,Ding J,Gao F,Xia G

    更新日期:2012-08-01 00:00:00

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    pub_type: 杂志文章


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    更新日期:2008-10-01 00:00:00

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    pub_type: 杂志文章


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    journal_title:International journal of laboratory hematology

    pub_type: 杂志文章


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    更新日期:2010-08-01 00:00:00

  • Establishment of reference interval for immature platelet fraction.

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    pub_type: 杂志文章


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    更新日期:2013-10-01 00:00:00

  • The clinical utility of new reticulocyte and erythrocyte parameters on the Sysmex XN 9000 for iron deficiency in pregnant patients.

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    pub_type: 临床试验,杂志文章


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    pub_type: 杂志文章


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    更新日期:2011-12-01 00:00:00

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    pub_type: 杂志文章


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    pub_type: 杂志文章


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    pub_type: 临床试验,杂志文章,多中心研究


    authors: Hermansen NE,Borup R,Andersen MK,Vangsted AJ,Clausen NT,Kristensen DL,Nielsen FC,Gimsing P

    更新日期:2016-06-01 00:00:00

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    pub_type: 杂志文章


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    更新日期:2016-04-01 00:00:00

  • Paediatric reference intervals for common coagulation assays in Chinese children as performed on the STA-R coagulation analyzer.

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    pub_type: 杂志文章


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    journal_title:International journal of laboratory hematology

    pub_type: 杂志文章


    authors: Mikula M,Buller-Burckle A,Gallivan M,Sun W,Franklin CR,Strom CM

    更新日期:2011-06-01 00:00:00

  • Discrepancies between APTT results determined with different evaluation modes on automated coagulation analyzers.

    abstract::Modern photo-optical coagulometers collect optical data in the form of reaction curves and offer a possibility to determine clotting times at different points of clot formation by using different evaluation modes. The objectives of this study were to determine the possible impact of an evaluation mode on activated par...

    journal_title:International journal of laboratory hematology

    pub_type: 杂志文章


    authors: Milos M,Herak DC,Zadro R

    更新日期:2010-02-01 00:00:00

  • IgG subclasses of FVIII inhibitors in an argentine cohort of severe hemophilia A patients: Analysis by flow cytometry.

    abstract:INTRODUCTION:FVIII inhibitors consist of a polyclonal population of antibodies. Previous studies have demonstrated different distribution of IgG subclasses. IgG4 was associated to high level of FVIII inhibitors and failure of immune tolerance induction (ITI) treatment. This study monitored the relative distribution of ...

    journal_title:International journal of laboratory hematology

    pub_type: 杂志文章


    authors: Irigoyen MB,Primiani L,Felippo M,Candela M,de Bracco MME,Galassi NV

    更新日期:2017-12-01 00:00:00

  • Low expression of TET2 gene in pediatric acute lymphoblastic leukemia is associated with poor clinical outcome.

    abstract:INTRODUCTION:TET2, a member of the Ten-Eleven translocation gene family, catalyzes the conversion of 5-methylcytosine to 5-hydroxymethylcytosine in DNA. Low expression of TET2 has been reported as a prognostic factor for several types of malignancies in adult patients. However, there have been few data on the effect of...

    journal_title:International journal of laboratory hematology

    pub_type: 杂志文章


    authors: Zhang P,Weng WW,Chen P,Zhang Y,Ruan JF,Ba DD,Xu WQ,Tang YM

    更新日期:2019-10-01 00:00:00

  • Auer rod-like intracytoplasmic inclusions in multiple myeloma. A case report and review of the literature.

    abstract::Several intracytoplasmic morphological changes in the plasma cells of multiple myeloma have been described previously. However, Auer rod-like inclusions are rarely found in these types of cells. Here, we report a case of multiple myeloma with Auer rod-like, needle-shaped intracytoplasmic inclusions in plasma cells. A ...

    journal_title:International journal of laboratory hematology

    pub_type: 杂志文章,评审


    authors: Hütter G,Nowak D,Blau IW,Thiel E

    更新日期:2009-04-01 00:00:00

  • Establishment of reference intervals for pediatric complete capillary blood counts: A multicenter study in Beijing.

    abstract:INTRODUCTION:Clinical reference intervals represent the normal range of clinical parameters for distinguishing healthy and sick individuals, and they show some variation among different populations. Many reference intervals are still lacking for the pediatric population in China. Thus, the aim of this study was to esta...

    journal_title:International journal of laboratory hematology

    pub_type: 杂志文章


    authors: Sun Q,Yue Y,Tian Y,Wang Q

    更新日期:2020-10-01 00:00:00

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    pub_type: 杂志文章,评审


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    更新日期:2015-05-01 00:00:00

  • The diagnostic and clinical impact of genetics and epigenetics in acute myeloid leukemia.

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    journal_title:International journal of laboratory hematology

    pub_type: 杂志文章,评审


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    abstract::Using previously described procedures, this study quantified T-cell, T-cell subset, B-cell and NK-cell populations with the CD-Sapphire haematology analyser in a series of patients with mild to moderate lymphocytosis. Lymphocyte counts ranged from 6.0 to 14.9 x 10(9)/l, with 86/97 being <10.0 x 10(9)/l. Immunophenotyp...

    journal_title:International journal of laboratory hematology

    pub_type: 杂志文章


    authors: Molero T,Lemes A,DE LA Iglesia S,Scott CS

    更新日期:2007-12-01 00:00:00

  • Hemostasis critical values among Iranian clinical laboratories "National Survey of 157 Clinical Laboratories".

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    pub_type: 杂志文章


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    abstract:INTRODUCTION:Women are at higher risk of venous thromboembolism (VTE) during pregnancy and the puerperium. Global coagulation assays (GCAs), including thromboelastography (TEG), thrombin generation using the calibrated automated thrombogram (CAT) and fibrin generation using the overall haemostatic potential assay (OHP)...

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    pub_type: 杂志文章


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    journal_title:International journal of laboratory hematology

    pub_type: 杂志文章,评审


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    pub_type: 杂志文章


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    pub_type: 杂志文章


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    更新日期:2014-08-01 00:00:00

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    journal_title:International journal of laboratory hematology

    pub_type: 杂志文章


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    更新日期:2008-06-01 00:00:00