불응성 혈전성혈소판감소자반증의 성공적인 저용량 리툭시맙 치료 1예
Successful Treatment with Low-dose Rituximab in a Patient with Plasma Exchange-refractory Idiopathic Thrombotic Thrombocytopenic Purpura
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초록
성인 혈전성혈소판감소자반증(thrombotic thrombocytopenic purpura, TTP)은 미세혈관병용혈빈혈(microangiopathic hemolytic anemia)과 혈소판감소증이 동반된 혈전미세혈관병증(thrombotic microangiopathy)을 특징으로 하며 신경학적 이상, 신기능장애, 발열 등이 동반될 수 있는 비교적 드문 혈액질환으로 a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member of 13 (ADAMTS-13)의 결핍 혹은 활성도 저하가 관여하는 것으로 알려져 있다[1]. 혈장교환술에불응하거나 재발한 성인 특발성 TTP의 경우 B세포 림프구의 표면 항원인 CD20에 대한 단일클론항체인 rituximab을 이용한 치료가 효과가 있음이 다수의 증례 및 소규모 연구를통하여 보고되어 있는데 대부분 1회 투약 시 375 mg/m2의 용량을 사용하였다[2-4]. 이는 림프계 종양 및 일부 자가면역질환에서 사용되는 통상적인 용량 및 용법에 따른 것으로 실제성인의 TTP에서 rituximab의 적절한 용량에 대하여 구체적으로 연구된 바는 없다. 저자들은 혈장교환술과 부신피질스테로이드 제제에 불응성인 성인의 특발성 TTP에서 상대적으로 적은 용량을 투여한 후 관해에 이르게 된 증례를 경험하였기에 이를 보고하고자 한다.
Abstract
Thrombotic thrombocytopenic purpura (TTP) is a clinical syndrome characterized by micro-angiopathic hemolytic anemia, thrombocytopenia, fever, renal disorders, and neurological manifestations. Its clinical course is rapid and the mortality rate is high if untreated or relapse occurs. Previous studies reported that rituximab, a monoclonal antibody for CD20 surface antigen on B lymphocytes, may be effective in treating idiopathic TTP that is refractory to plasma exchange or relapses after remission. A 27-year-old Vietnamese man presented with fever and fatigue starting 3 days earlier, which was diagnosed as idiopathic TTP. To overcome his poor responsiveness to combined therapy using steroids and plasma exchange, rituximab was considered. In the current case, the patient was treated with a lower dose of rituximab, instead of the conventional 375 mg/m2/week, and achieved successful remission.
참고문헌 (10)
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