Rituximab provided long-term remission in a patient with severe thrombotic thrombocytopenic purpura refractory to plasma exchange

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  • Rituximab投与が奏効し, 長期寛解を維持している血漿交換抵抗性重症血栓性血小板減少性紫斑病
  • 症例報告 第154回日本臨床血液学会例会:三浦修例会長 推薦演題 Rituximab投与が奏効し,長期寛解を維持している血漿交換抵抗性重症血栓性血小板減少性紫斑病
  • ショウレイ ホウコク ダイ154カイ ニホン リンショウ ケツエキ ガッカイ レイカイ ミウラオサムレイカイチョウ スイセン エンダイ Rituximab トウヨ ガ ソウコウ シ チョウキカンカイ オ イジ シテ イル ケッショウ コウカン テイコウセイ ジュウショウ 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

We report a patient with severe thrombotic thrombocytopenic purpura (TTP) refractory to plasmapheresis who was successfully treated with rituximab. A 57-year-old male patient was referred to our department for further differential diagnosis and treatment of anemia and severe thrombocytopenia. Progressive psychoneurotic symptoms, hemolytic anemia, thrombocytopenia, renal function insufficiency and fever led us to the diagnosis of TTP. ADAMTS13 activity was below 3% and an inhibitor for ADAMTS13 was detected. Treatment with plasmapheresis and high-dose steroid was initiated but without clinical benefit. Two weeks following the initiation of plasmapheresis, we decided to treat the patient with 7 cycles of rituximab. No severe rituximab-related adverse effects were observed. After treatment with rituximab, the disease remitted, and the ADAMTS13 activity level increased. The patient has remained in complete remission for more than 1 year. Our data suggest that rituximab may be the optimal immunosuppressive therapy for refractory thrombotic thrombocytopenic purpura caused by an anti-ADAMTS 13 inhibitor.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 47 (12), 1528-1532, 2006

    The Japanese Society of Hematology

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