Successful treatment of thrombotic thrombocytopenic purpura with repeated plasma exchange in a patient with microscopic polyangitis
Koji Nagai1 , Takuya Kotani1 , Tohru Takeuchi1 , Takeshi Shoda1 , Ayu Hata-Kobayashi1 , Daisuke Wakura1 , Maki Kagitani1 , Shigeki Makino1 , Toshiaki Hanafusa1
8 May 2008
30 June 2008
2 September 2008
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Thrombotic thrombocytopenic purpura (TTP) is in rare cases associated with antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, and often has a fatal outcome. We report the case of a 77-year-old woman with microscopic polyangitis (MPA) presenting with TTP. Rapidly progressive renal dysfunction and paralysis and sensory disturbance of the left lower limb were noted. Serum creatinine was 3.95 mg/dl, and the titer of myeloperoxidase-ANCA was 238 EU. She was diagnosed with MPA, and high-dose methylprednisolone was initiated, followed by 60 mg/day of prednisolone. Hemolytic anemia with red blood cell fragmentation, purpura, and thrombocytopenia developed during the course of active MPA. The activity of disintegrin and metalloproteinase with thrombospondin type 1 motifs 13 (ADAMTS13) was moderately decreased (27%). She was diagnosed with TTP, and plasma infusion was initiated, followed by plasma exchange (PE) with 40 units of fresh frozen plasma. Thrombocytopenia continued for more than a month (5-10 × 104/µl). PE was repeatedly performed two or three times a week during the first 8 weeks from the beginning of PE in addition to prednisolone. Her clinical and laboratory findings gradually improved, and ADAMTS13 activity increased to 68%. The findings in this case suggested that ANCA-associated vasculitis may be involved in the development and the pathogenesis of TTP, and that repeated PE may need to be performed in addition to immunosuppressive therapy.
ADAMTS13 - Microscopic polyangitis - Plasma exchange - Thrombotic thrombocytopenic purpura