A Splenectomy in Plasma Exchange Refractory Thrombotic Thrombocytopenia Purpura / 대한진단검사의학회지

ABSTRACT

Thrombotic thrombocytopenic purpura (TTP) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, and variable abnormalities in renal function and mental status. The pathogenesis of TTP is related to an inhibitor or deficiency of the von Willebrand factor (vWF)-cleaving protease (a disintegrin and metalloprotease with thrombospondin type 1 repeats; ADAMTS-13) that cleaves the large vWF multimers. Uncleaved, large vWF molecules are present in TTP and induce thrombosis in small vessels. Even though plasma exchange was proven effective in TTP, 20-40% of the cases showed refractory to plasma exchange. We describe a 41 years old female with plasma exchange refractory TTP who was completely recovered from anemia, thrombocytopenia, and accompanying symptoms following splenectomy.