Pediatria pre prax 6/2013

When thrombocytopenia is not immune thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (syndrome Moschcowitz, TTP) is a very rare hematological disease in childhood and adolescent age with incidence 1–4 cases/1 milion persons/year. It is characterized by reversible aggregation of thrombocytes in microcirculation, whitch results in tissue and organ ischemia. Clinical symptoms are: skin and mucosal bleeding, anemic syndrome, neurological symptoms, fever and renal involvement. In most cases, TTP starts with rapid onset of symptoms, whitch get worse dramatically in a few hours and with inappropriate treatment can result in organ failure and death. In our case report we present a patient, 17 years old girl, with atypical relapsing thrombotic thrombocytopenic purpura with severe complications.

Keywords: thrombotic thrombocytopenic purpura, thrombocytopenia, hemolytic anemia, plasma exchange therapy.