Refractory thrombotic thrombocytopenic purpura successfully treated with a combination of rituximab and vincristine

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  • Rituximabとvincristineの併用が奏効した難治性血栓性血小板減少性紫斑病
  • 症例報告 Rituximabとvincristineの併用が奏効した難治性血栓性血小板減少性紫斑病
  • ショウレイ ホウコク Rituximab ト vincristine ノ ヘイヨウ ガ ソウコウ シタ ナンジセイ ケッセンセイ ケッショウバン ゲンショウセイ シハンビョウ

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Abstract

A 26-year-old man was referred to our hospital with vomiting and high fever. He was disoriented and laboratory results showed microangiopathic hemolytic anemia (Hb 7.1 g/dl) and severe thrombocytopenia (15×103/μl). The diagnosis of thrombotic thrombocytopenic purpura (TTP) was established. The activity of von Willebrand cleaving protease (ADAMTS13) was found to be remarkably low (<0.5%) and the high activity of the inhibitor (11.0 Bethesda U/ml) was detected, confirming the diagnosis of typical acquired TTP. Although he had been successfully treated with daily plasma exchange and methylprednisolone, he relapsed after a week. To this therapeutic strategy we added four weekly doses of rituximab (375 mg/m2) and two weekly doses of vincristine (1 mg/m2) simultaneously. The response was very rapid and complete, that is, the platelet count recovered to normal seven days after the first rituximab and vincristine treatment. The patient maintains complete remission nine months later under the administration of 17.5 mg prednisolone. Recovery of the plasma ADAMTS13 activity was clearly correlated with the decrease or disappearance of the inhibitor activity. Combination of rituximab and vincristine therapy would appear to be very effective against refractory TTP.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 48 (2), 144-147, 2007

    The Japanese Society of Hematology

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