Korean J Nephrol.
2008 Mar;27(2):243-247.
A Case of Splenectomy in a Patient with Refractory Thrombotic Thrombocytopenic Purpura
- Affiliations
-
- 1Department of Internal Medicine, College of Medicine, Korea University, Seoul , Korea. yjkwon@korea.ac.kr
- 2Department of Internal Medicine, Wonkwang University School of Medicine, Gunpo, Korea.
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.