title: High-dose immunoglobulin infusion for thrombotic thrombocytopenic purpura refractory to plasma exchange and steroid therapy.
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.
authors.affiliation: Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea.
authors.forename: Seh Jong
authors.initials: SJ
authors.lastname: Park
authors.forename: Seok Jin
authors.initials: SJ
authors.lastname: Kim
authors.forename: Hee Yun
authors.initials: HY
authors.lastname: Seo
authors.forename: Moon Ju
authors.initials: MJ
authors.lastname: Jang
authors.forename: Doyeun
authors.initials: D
authors.lastname: Oh
authors.forename: Byung Soo
authors.initials: BS
authors.lastname: Kim
authors.forename: Jun Suk
authors.initials: JS
authors.lastname: Kim
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D006801:Humans; D007136:Immunoglobulins / Q000008:administration & dosage / Q000627:therapeutic use*; D008297:Male; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000188:drug therapy*; D016879:Salvage Therapy; D055502:Secondary Prevention; D017211:Treatment Failure
chemical_list: D000305:Adrenal Cortex Hormones; D007136:Immunoglobulins
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