Korean J Intern Med.  2008 Sep;23(3):161-164. 10.3904/kjim.2008.23.3.161.

High-dose Immunoglobulin Infusion for Thrombotic Thrombocytopenic Purpura Refractory to Plasma Exchange and Steroid Therapy

• Park SJ
• Kim SJ
• Seo HY
• Jang MJ
• Oh D
• Kim BS
• Kim JS

• KMID: 759975
• DOI: http://doi.org/10.3904/kjim.2008.23.3.161

Abstract

The outcomes of the treatment of thrombotic thrombocytopenic purpura (TTP) have been shown to be improved by the administration of plasma exchange. However, treatment options are currently limited for cases refractory to plasma exchange. The autoantibodies that block the activity of ADAMTS13 have been demonstrated to play a role in the pathogenesis of TTP; therefore, high-dose immunoglobulin, which can neutralize these autoantibodies, may be useful for refractory TTP. However, successful treatment with high-dose immunoglobulin for TTP refractory to plasma exchange and corticosteroids has yet to be reported in Korea. Herein, we describe a refractory case which was treated successfully with high-dose immunoglobulin. A 29-year-old male diagnosed with TTP failed to improve after plasma exchange coupled with additional high-dose corticosteroid therapy. As a salvage treatment, we initiated a 7-day regimen of high-dose immunoglobulin (400 mg/kg) infusions, which resulted in a complete remission, lasting up to the last follow-up at 18 months. High-dose immunoglobulin may prove to be a useful treatment for patients refractory to plasma exchange; it may also facilitate recovery and reduce the need for plasma exchange.

Keyword

Thrombotic Thrombocytopenic Purpura; Plasma Exchange; Glucocorticoids; Immunoglobulin

MeSH Terms

Adrenal Cortex Hormones/*therapeutic use
Adult
Humans
Immunoglobulins/administration & dosage/*therapeutic use
Male
*Plasma Exchange
Purpura, Thrombotic Thrombocytopenic/*drug therapy
Recurrence/prevention & control
Salvage Therapy
Treatment Failure