[Thrombotic thrombocytopenic purpura in pregnancy. Case report]. / Trombotická trombocytopenická purpura v tehotenství. Kazuistika.

OBJECTIVE: Description of case of patient with rare thrombotic thrombocytopenic purpura in pregnancy. SUBJECT: Case report. SETTING: Department of Gynecology and Obstetrics, Charles University and University Hospital Motol, Prague. CONCLUSION: Thrombotic thrombocytopenic purpura (TTP) is a rare and substantial disorder characterized with combination of microangiopathic haemolytic anemia, consumption trombocytopenia and symptoms of organs dysfunction--especially kidneys and neurological deficiency. It's caused by production of microthrombi affecting small blood vessels. These palatelets-rich microtrombi are formed due to deficiency of the enzyme ADAMTS13--metalloprotease which is responsible for cleaving of ultralarge multimers of von Willebrand factor into smaller units. In our case report we describe patient with TTP in pregnancy. Therapy with corticosteroids and immunoglobulines was not effective, improvement of thrombocytopenia appeared after plasmapheresis (total count 14). The delivery was induced at term without complications. Target examination confirmed diagnosis of secondary TTP.