The thrombocytopenia of WAS: a familial form of ITP?
In the first report of the concurrent immunodeficiency, thrombocytopenia, and eczema that we now call the Wiskott-Aldrich Syndrome (WAS), Alfred Wiskott asked whether it could be a familial form of Werlhof's disease (now called ITP). This review summarizes what is known about platelet production, consumption, and function in clinical and murine WAS. Both platelet production and consumption are affected by WASP deficiency. Likely molecular mechanisms have been identified for the former process, but remain problematic for the latter. Recent data in a murine model suggest that WASP deficiency could increase both the incidence of antiplatelet antibodies and susceptibility to their enhancement of platelet consumption. Wiskott's original speculation about the relationship between WAS and ITP may need to be reconsidered.
Pathology and Laboratory Medicine Service, Memphis VA Medical Center, Department of Pathology and Laboratory Medicine, University of Tennessee Health Sciences Center, Memphis, TN, USA. tstrom@utmem.edu
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D000818:Animals; D001792:Blood Platelets / Q000276:immunology / Q000502:physiology*; D006801:Humans; D008533:Megakaryocytes / Q000276:immunology / Q000502:physiology*; D051379:Mice; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000276:immunology* / Q000503:physiopathology; D013921:Thrombocytopenia / Q000276:immunology*; D034061:Thrombopoiesis / Q000276:immunology; D014923:Wiskott-Aldrich Syndrome / Q000276:immunology* / Q000503:physiopathology