Experience of successful diagnosis and first-stage treatment of a patient with Moschcowitz syndrome

Abstract

Thrombotic thrombocytopenic purpura (TTP), also known as Moschcowitz syndrome, is an orphan, life-threatening, acute-onset disease caused by a deficiency of the metalloprotease that cleaves extra large multimers of von Willebrand factor (ADAMTS13). TTP is a difficult pathology to diagnose due to the pronounced polymorphism of symptoms. In this article, a clinical case of successful diagnosis and first stage therapy of a patient with laboratory-confirmed TTP is presented. Afterwards, the patient was safely transported to the National Medical Research Center for Hematology (Moscow).