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KMID : 0359920080270020243
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Korean Journal of Nephrology
2008 Volume.27 No. 2 p.243 ~ p.247
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A Case of Splenectomy in a Patient with Refractory Thrombotic Thrombocytopenic Purpura
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Lee Jae-Won
Kim Sun-Chul
Oh Se-Won
Cha Jin-Joo
Kim Hye-Won
Boo Chang-Su
Lee Ji-Eun
Kwon Young-Joo
Pyo Heui-Jung
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Abstract
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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.
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KEYWORD
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Thrombotic thrombocytopenic purpura, Splenectomy
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