Inherited thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder characterized by thrombocytopenia and microangiopathic hemolytic anemia accompanied by variable neurological dysfunction, kidney failure, and fever.

It is caused by severely reduced activity of the von Willebrand factor-cleaving protease ADAMTS13.

Hereditary TTP, caused by ADAMTS13 gene mutations, is much less common.

[1] This article incorporates text by Romão de Souza et al. available under the CC BY 4.0 license.

This genetic disorder article is a stub.