作者: Gallia G. Levy , David G. Motto , David Ginsburg
DOI: 10.1182/BLOOD-2004-10-4097
关键词: Thrombocytopenic purpura 、 Von Willebrand factor 、 Hemostasis 、 Immunology 、 Thrombotic thrombocytopenic purpura 、 ADAMTS13 、 Thrombospondin 、 Medicine 、 Hematologic disease 、 Pathogenesis
摘要: It has now been 3 years since the von Willebrand factor (VWF)–cleaving protease implicated in thrombocytopenic purpura (TTP) pathogenesis was identified as ADAMTS13 (adisintegrin-like and metalloprotease with thrombospondin type 1 motif 13). More than 50 mutations resulting familial TTP have reported. Considerable progress also realized toward understanding role of normal hemostasis, well mechanisms by which deficiency contributes to pathogenesis. Measurement activity other pathologic conditions remains a focus substantial clinical research effort. Building on these studies, continued investigation VWF holds considerable promise for advancing should lead improved diagnosis treatment this important hematologic disease.