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INTRINSIC PLATELET DEFECTS IN HEREDITARY THROMBOCYTOPENIA *
Author(s) -
Murphy Scott
Publication year - 1972
Publication title -
annals of the new york academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1111/j.1749-6632.1972.tb16314.x
Subject(s) - splenectomy , wiskott–aldrich syndrome , platelet , disease , medicine , thrombocytopenic purpura , immunology , inheritance (genetic algorithm) , genetics , biology , pathology , spleen , gene
SUMMARY Nine families with hereditary thrombocytopenia have been described. They have been discussed and classified according to mode of inheritance, the presence or absence of clinical features of the Wiskott‐Aldrich Syndrome, platelet size, and platelet lifespan studies. These features and an assay for serum Antiplatelet Factor help to distinguish hereditary disease from acquired thrombocytopenias such as idiopathic thrombocytopenic purpura. This distinction is important, since response to therapy, particularly splenectomy, is clearly different for hereditary and acquired disease.