A Case of Splenectomy in a Patient with Refractory Thrombotic Thrombocytopenic Purpura.
- Author:
Jae Won LEE
1
;
Sun Chul KIM
;
Se Won OH
;
Jin Joo CHA
;
Hye Won KIM
;
Chang Su BOO
;
Ji Eun LEE
;
Young Joo KWON
;
Heui Jung PYO
Author Information
1. Department of Internal Medicine, College of Medicine, Korea University, Seoul , Korea. yjkwon@korea.ac.kr
- Publication Type:Case Report
- Keywords:
Thrombotic thrombocytopenic purpura;
Splenectomy
- MeSH:
Antibodies, Monoclonal, Murine-Derived;
Cyclosporine;
Humans;
Plasma;
Plasma Exchange;
Plasmapheresis;
Platelet Count;
Purpura, Thrombotic Thrombocytopenic;
Splenectomy;
Survival Rate;
Thymine Nucleotides;
Vincristine;
Rituximab
- From:Korean Journal of Nephrology
2008;27(2):243-247
- CountryRepublic of Korea
- Language:Korean
-
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.
