Resumo

SALAZAR MORALES, Miguel Fernando  e  REYES CASTRO, María Magdalena. Thrombotic thrombocytopenic purpura: A case and review of its physiopathology. Rev. Fac. Med. (Méx.) [online]. 2013, vol.56, n.1, pp.30-38. ISSN 2448-4865.

We report a case compatible with thrombotic thrombocytopenic purpura, autopsy findings and make a brief review of the literature. 19 year old woman with HELLP syndrome in her previous pregnancy who presented with neurological signs, thrombocytopenia and microangiopathic haemolytic anemia until her pass away fifteen days after being admitted to the hospital. Autopsy findings showed multiple thrombi in small sized vessels of several organs. Thrombotic thrombocitopenic purpura is a rare disease with a morphological expression featured of many microthrombi in the terminal arterioles of several vital structures. Moschcowitz was the first to inform multiple hyaline thrombi as the primordial finding of a partial autopsy case. He proposed that "a powerful poison with both agglutinative and hemolytic properties" was the causative agent but it was identified years later as unusually large fragments of Von Willebrand factor caused by a deficiency of ADAMTS13, a newly discovered metalloproteinase.

Palavras-chave : Thrombotic Thrombocitopenic Purpura; Thrombotic Microangiopathy; Shear Stress; Von Willebrand Factor; ADAMTS13.