비장적출술로 호전된 불응성 혈전성 혈소판감소성 자반증 1예

Abstract

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.