The Thrombocytopenia of WAS: a Familial Form of ITP?

Overview

Journal Immunol Res

Specialty Allergy & Immunology

Date 2008 Oct 16

PMID 18854955

Citations 8

Authors

Ted S Strom

Affiliations

Soon will be listed here.

Abstract

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.

Citing Articles

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