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DOI: 10.1055/s-0033-1333750
Thrombotic Thrombocytopenic Purpura in a 15-Year-Old Female
Thrombotische thrombozytopenische Purpura bei einer 15-jährigen PatientinPublication History
Publication Date:
21 March 2013 (online)
Introduction
Thrombotic thrombocytopenic purpura (TTP), first described in 1924 by Moschowitz, affects mainly adults, often those undergoing treatment for different types of cancer, infections, or after tissue transplantation (Sadler JE et al. Hematology Am Soc Hematol Educ Program 2004: 407–423). The clinical signs of TTP include anemia, thrombocytopenia, fever, renal damage, and cerebral symptoms like headaches, confusion, convulsions or coma.
TTP is classified into the group of microangiopathic hemolytic anemias similar to the hemolytic uremic syndrome, and is characterized by kidney damage with acute renal failure, anemia and thrombocytopenia (Dierkes F et al. PLoS One. 2012; 7: e30886. Epub 2012 Jan 23). An acquired autoimmune or an inherited deficiency of a von Willebrand Factor-cleaving plasma metalloprotease named ADAMTS-13 has been identified as the cause of TTP (Furlan M et al. Blood. 1997; 89: 3097–3103).
Here, we describe the case of a 15-year-old girl with idiopathic TTP that was treated effectively with plasmapheresis and corticosteroids.