Inherited Thrombotic Thrombocytopenic Purpura Revealed by Recurrent Strokes in a Male Adult: Case Report and Literature Review

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy related to a severe deficiency of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13). In this article, we describe the first case of a young male adult suffering from a hereditary TTP revealed by recurrent strokes, relapsing despite antiplatelet and anticoagulant therapy. Because of the persistent moderate thrombocytopenia, plasmatic ADAMTS13 activity was investigated and was found lower than 5% in the absence of anti-ADAMTS13 IgG.

Source: Journal of Stroke and Cerebrovascular Diseases - March 27, 2019 Category: Neurology Authors: Diane Beauvais, Laura Venditti, Olivier Chassin, B érangère Joly, Alain Ameri, Pierre Boisseau, Olivier Lambotte, Paul Coppo, Agnès Veyradier, Christian Denier Source Type: research