title: Successful treatment of refractory thrombotic thrombocytopenic purpura with cyclosporine and corticosteroids in a patient with systemic lupus erythematosus and antibodies to ADAMTS13.
abstract: A 46-year-old woman with systemic lupus erythematosus was hospitalized for purpura, hematochezia and hematuria. One week after admission, she developed grand mal seizures and coma and was diagnosed with thrombotic thrombocytopenic purpura (TTP) when fragmented red cells were found on the peripheral blood smear. Laboratory findings showed severe ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 repeats) deficiency and anti-ADAMTS13 antibodies, which in recent reports have indicated a poor prognosis. She was refractory to methylprednisolone pulse therapy and plasma exchange, but administration of cyclosporine induced remission without adverse effects. We propose that cyclosporine may be an effective treatment for cases of refractory TTP.
authors.affiliation: Clinical Immunology, Advanced Biomedical Applications, Graduate School of Comprehensive Human Sciences, University of Tsukuba.
authors.forename: Terukazu
authors.initials: T
authors.lastname: Enami
authors.forename: Takeshi
authors.initials: T
authors.lastname: Suzuki
authors.forename: Satoshi
authors.initials: S
authors.lastname: Ito
authors.forename: Ai
authors.initials: A
authors.lastname: Yoshimi
authors.forename: Makoto
authors.initials: M
authors.lastname: Sugihara
authors.forename: Mizuko
authors.initials: M
authors.lastname: Mamura
authors.forename: Taichi
authors.initials: T
authors.lastname: Hayashi
authors.forename: Daisuke
authors.initials: D
authors.lastname: Goto
authors.forename: Isao
authors.initials: I
authors.lastname: Matsumoto
authors.forename: Akito
authors.initials: A
authors.lastname: Tsutsumi
authors.forename: Takayuki
authors.initials: T
authors.lastname: Sumida
mesh_terms: D051722:ADAM Proteins / Q000276:immunology*; D000917:Antibody Formation / Q000276:immunology*; D001774:Blood Chemical Analysis; D016572:Cyclosporine / Q000627:therapeutic use*; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D008775:Methylprednisolone / Q000627:therapeutic use*; D008875:Middle Aged; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D012008:Recurrence; D018570:Risk Assessment; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D016572:Cyclosporine; D051722:ADAM Proteins; D008775:Methylprednisolone