F. Peyvandi
Abstract
Thrombotic microangiopathies (TMAs) are a wide spectrum of diseases characterized by thrombocytopenia, microangiopathic hemolytic anemia and widespread ischemic damage due to microvascular thrombosis. TMAs could be life-threatening unless promptly recognized and treated, hence demand a rapid differential diagnosis and initiation of proper therapy, especially in the light of the new targeted therapies available nowadays. Thrombotic thrombocytopenic purpura (TTP) is a rare and severe primary TMA, caused by the congenital or acquired deficiency of ADAMTS13, the von Willebrand factor-cleaving protease.
The severity of TTP, the significant recent developments in treatment and the heterogeneity of management worldwide, among other reasons, prompted the International Society of Thrombosis and Hemostasis (ISTH) to promote the draw up of evidence-based guidelines for the diagnosis and treatment of TTP.
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