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Light chain-restricted autoantibodies in chronic idiopathic thrombocytopenic purpura, but no evidence for circulating clonal B-lymphocytes

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Abstract

In chronic idiopathic thrombocytopenic purpura (ITP) platelet destruction is caused by antibodies directed against platelet membrane glycoproteins (GP), and the predominant autoantigens are known to be GPIb/IX and GPIIb/IIIa. In a recent study we reported that these antibodies frequently had a restricted light chain phenotype, thereby supporting a clonal origin. Similar findings and the presence of clonal B-cell populations in immune thrombocytopenias have been reported by others. In the present study we further explored the hypothesis of clonal B-cell expansions in chronic ITP. Twenty patients with chronic ITP were investigated. Antibodies were detected with an ELISA (MAIPA) specific for GPIb/IX and GPIIb/IIIa; circulating clonal B lymphocytes were assessed by flow-cytometric (FACE) clonal-excess analysis and by analyzing Ig-gene rearrangements (CDR3) with the PCR technique. Nine patients displayed a GP-specific antibody restricted to either kappa or lambda phenotype. However, FACS analysis and Ig-gene rearrangement studies did not disclose any circulating clonal B-cell population. Considering the sensitivity of the FACS analysis and Ig-gene rearrangement for detection of clonal B-cell populations, the hypothesis of clonally derived autoantibodies in ITP is still valid. Most probably, the clonal B-cell expansion responsible for the production of autoantibodies in ITP, if present, is below the detection limit for the techniques employed.

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Authors and Affiliations

  1. Section of Hematology, Department of Medicine, Sahlgrenska Hospital, S-413 45, Göteborg, Sweden

    D. Stockelberg, M. Hou, J. Kutti & H. Wadenvik

  2. Department of Clinical Chemistry and Transfusion Medicine, Sahlgrenska University Hospital, University of Göteborg, Sweden

    S. Jacobsson

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  1. D. Stockelberg
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  2. M. Hou
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  3. S. Jacobsson
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  4. J. Kutti
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  5. H. Wadenvik
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Stockelberg, D., Hou, M., Jacobsson, S. et al. Light chain-restricted autoantibodies in chronic idiopathic thrombocytopenic purpura, but no evidence for circulating clonal B-lymphocytes. Ann Hematol 72, 29–34 (1996). https://doi.org/10.1007/BF00663013

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  • DOI: https://doi.org/10.1007/BF00663013

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