title: Intravenous gamma globulin for thrombotic microangiopathy of unknown etiology.
abstract: We encountered the case of a 4-year-old boy with thrombotic microangiopathy (TMA) of unknown etiology. Verotoxin-induced hemolytic uremic syndrome (HUS), Streptococcus-pneumoniae-related HUS, factor H deficiency, drug-induced thrombotic thrombocytopenic purpura (TTP), and ADAMTS13 (von Willebrand factor-cleaving protease; a disintegrin-like and metalloprotease with thrombospondin type 1 repeats)-related TTP were excluded. His condition was refractory to anticoagulants and plasma exchange, and his clinical course was catastrophic, with central nervous system symptoms and progressive renal failure. However, factual treatment of intravenous gamma globulin (IVIG) ended the hemolysis and resulted in a rise in platelet count. He fully recovered except for end-stage renal failure, but he underwent a successful renal transplant after peritoneal dialysis. He has not suffered a relapse of TMA or an allograft rejection for 4 years. IVIG might be an option for some patients with TMA of unknown etiology refractory to conventional treatment.
authors.affiliation: Children's Medical Center, Yokohama City University Medical Center, 4-57, Urafune-cho, Minami-ku, Yokohama, 232-0024, Japan. hi5-si@urahp.yokohama-cu.ac.jp
authors.forename: Shuichi
authors.initials: S
authors.lastname: Ito
authors.forename: Kenichi
authors.initials: K
authors.lastname: Okuyama
authors.forename: Tomoko
authors.initials: T
authors.lastname: Nakamura
authors.forename: Jun-Ichi
authors.initials: J
authors.lastname: Tetanishi
authors.forename: Kazuo
authors.initials: K
authors.lastname: Saito
authors.forename: Masanori
authors.initials: M
authors.lastname: Matsumoto
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Fujimura
authors.forename: Yukoh
authors.initials: Y
authors.lastname: Aihara
authors.forename: Shumpei
authors.initials: S
authors.lastname: Yokota
mesh_terms: D001706:Biopsy; D002675:Child, Preschool; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007668:Kidney / Q000098:blood supply / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000601:surgery; D016030:Kidney Transplantation; D008297:Male; D011379:Prognosis; D013927:Thrombosis / Q000188:drug therapy* / Q000209:etiology*; D014652:Vascular Diseases / Q000188:drug therapy* / Q000209:etiology*
chemical_list: D016756:Immunoglobulins, Intravenous