The thrombocytopenia of WAS: a familial form of ITP?
| Autor: | Ted S. Strom |
|---|---|
| Rok vydání: | 2008 |
| Předmět: |
Blood Platelets
Allergy Immunology macromolecular substances Disease Thrombopoiesis Mice hemic and lymphatic diseases Platelet production Animals Humans Medicine Platelet Immunodeficiency Purpura Thrombocytopenic Idiopathic Familial form biology business.industry medicine.disease Thrombocytopenia Wiskott-Aldrich Syndrome Murine model biology.protein Antibody business Megakaryocytes |
| Zdroj: | Immunologic Research. 44:42-53 |
| ISSN: | 1559-0755 0257-277X |
| DOI: | 10.1007/s12026-008-8069-2 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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