비장적출술로 호전된 불응성 혈전성 혈소판감소성 자반증 1예

• Published in: Kidney research and clinical practice pp. 243 - 247
• Main Authors: 이재원, 김선철, 오세원, 차진주, 김혜원, 부창수, 이지은, 권영주, 표희정
• Format: Journal Article
• Language: Korean
• Published: 대한신장학회 01.03.2008
• Subjects: 내과학
• ISSN: 2211-9132; 2211-9140

The introduction of plasma exchange has significantly improved the outcome of thrombotic thrombocytopenic purpura (TTP) and the survival rate was increased from 10 to 80-90%. TTP refractory to plasma exchange therapy, however, is still a therapeutic challenge. We describe here a patient who partially responded to plasma exchange therapy, but remained dependent on plasma infusions. To discontinue plasma therapy, several attempts using agents such as rituximab, vincristine, and cyclosporine A had been tried, but all failed. After splenectomy, serum LDH and blood platelet count were normalized. Plasmapheresis were we able to discontinue after 2 weeks of splenectomy. Steroid and cyclosporine were tapered off after 3 months and 5 months after splenectomy respectively, and the patient has been staying in remission ever since. We suggest that splenectomy is a worthwhile treatment option in patients with refractory TTP. KCI Citation Count: 0