재발 혈전저혈소판혈증자색반병에서 Rituximab을 이용한 치료 경험 1예 (A Case Report of Rituximab Therapy for Recurrent Thrombotic Thrombocytopenia Purpura)

서지정보

· 발행기관 : 대한혈액학회
· 수록지 정보 : Blood Research / 44권 / 3호 / 193 ~ 197페이지
· 저자명 : 이승범, 최윤숙, 이제환, 천윤희, 유지희, 최원정, 정상수

초록

이전에 건강하였던 38세 남성이 혈소판감소 및 의식 저하를 주소로 내원하여 혈전저혈소판혈증자색반병으로 진단받고 혈장교환술 치료를 시행 받았다. 반복적인 혈장교환술 치료 및 고용량 겉질스테로이드 투여에도 반복적으로 재발하는 경과를 보였다. 재발 혈전저혈소판혈증자색반병으로 진단하여 림프구 CD20 항원에 대한 단클론항체인 rituximab을 375 mg/㎡ 용량으로 1주 간격으로 4회 투여하였다. 이후 혈소판이 회복되고 젖산탈수소효소가 감소하였으며, 혈장교환술 및 겉질스테로이드 치료의 중단 후에도 혈전저혈소판혈증자색반병의 재발 없이 외래 추적관찰 중이다.

영어초록

Thrombotic thrombocytopenic purpura (TTP) is a rare medical condition characterized by thrombocytopenia, microangiopathic hemolytic anemia, neurologic manifestations, and infrequently, renal involvement. In many cases, TTP is associated with deficiency of the von Willebrand factor-cleaving protease, ADAMTS13, and treatment with plasma exchange is effective in the majority of patients. We report a patient with acute TTP in whom remission was not achieved by initial treatment consisting of plasma exchange and corticosteroids. In view of the severe autoantibody-mediated ADAMTS-13 deficiency, treatment was initiated with rituximab, a chimeric monoclonal antibody directed against the CD 20 antigen present on B lymphocytes. The patient received 4 weekly infusions of rituximab (375 mg/㎡). Four weeks after the last infusion of rituximab, a complete clinical and laboratory remission was documented. We conclude that rituximab should be considered in patients with TTP with acquired ADAMTS-13 deficiency, who fail to respond to standard treatment with plasma exchange and corticosteroids. Rituximab may result in a lowered requirement for plasmapheresis and avoid the complications of salvage immunosuppressive therapy.

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