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.

Keyword

Thrombotic thrombocytopenic purpura; Splenectomy

MeSH Terms

Antibodies, Monoclonal, Murine-Derived
Cyclosporine
Humans
Plasma
Plasma Exchange
Plasmapheresis
Platelet Count
Purpura, Thrombotic Thrombocytopenic
Splenectomy
Survival Rate
Thymine Nucleotides
Vincristine
Rituximab
Antibodies, Monoclonal, Murine-Derived
Cyclosporine
Thymine Nucleotides
Vincristine
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