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Hereditary Autoimmune Thrombocytopenic Purpura: An Immunologic and Genetic Study

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▸Requests for reprints should be addressed to Simon Karpatkin, M.D.; New York University Medical Center, 550 First Avenue; New York, NY 10016.

New York University Medical Center, New York Blood Center, and the Rockefeller University; New York, New York

Ann Intern Med. 1981;94(6):781-782. doi:10.7326/0003-4819-94-6-781
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Although numerous cases of hereditary thrombocytopenia have been reported (1), only four families (1, 2) have clinical findings consistent with the diagnosis of autoimmune thrombocytopenic purpura. None of these had antiplatelet antibody measurements of their platelets. In this report we document the first case of familial autoimmune thrombocytopenic purpura with bound platelet antibody. This family provided a unique opportunity to ascertain the immunogenetics in six related patients, four of whom developed autoimmune thrombocytopenic purpura. The mode of inheritance and potential mechanisms of action of the histocompatibility determinants that influence disease were examined. The autoimmune thrombocytopenic purpura in this family segregated


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