Management of Thrombotic Thrombocytopenic Purpura with Autoantibodies to ADAMTS-13 and Concurrent Preeclampsia in Pregnancy: Multidisciplinary Team Approach

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare, severe life-threatening emergency characterized by microangio-pathic hemolytic anemia, thrombocytopenia, acute renal insufficiency, altered mental status, and fever. It exists in both congenital and acquired forms and is associated with the absence or severe depletion of von Willebrand factor cleaving protease known as a disintegrin and metalloproteinase with thrombospondin-like repeats (ADAMTS-13). 1,2 A deficiency in this protease can be congenital or the result of an acquired auto antibody to ADAMTS-13 and can lead to extensive platelet adhesion and clumping and possibly secondary end-organ damage. Plasma-based therapy containing re-placements of ADAMTS-13 was a major advancement for treatment of TTP. Today, high-dose intravenous methylpred-nisolone and plasma exchange are the mainstay treatments. Pregnancy can precipitate the disease in first-time patients or can exacerbate its recurrence. However, if TTP occurs for the first time during pregnancy, it may mimic other severe