title: Thrombotic thrombocytopenic purpura associated with pegylated-interferon alpha-2a by an ADAMTS13 inhibitor in a patient with chronic hepatitis C.
abstract: A deficiency of ADAMTS13 leads to platelet clumping and/or thrombi formation, finally resulting in thrombotic thrombocytopenic purpura (TTP). In this study, a 62-year-old male with chronic hepatitis C developed TTP a month after long-term pegylated-interferon (PEG-IFN) treatment. The observed low level of activity of plasma ADAMTS13 following PEG-IFN treatment was shown to gradually increase with the improvement of TTP, while the titer of an inhibitory anti- ADAMTS13 IgG antibody decreased concomitant with the increase in ADAMTS13 activity. Serial determination of ADAMTS13 activity and its inhibitor may provide useful information for the diagnosis and treatment of IFN-associated TTP, as well as its pathogenesis.
authors.affiliation: Department of Internal Medicine, Matsumoto National Hospital,Matsumoto, Japan. kiyoshikitano@mac.com
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Kitano
authors.forename: Yukio
authors.initials: Y
authors.lastname: Gibo
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kamijo
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Furuta
authors.forename: Satohiro
authors.initials: S
authors.lastname: Oguchi
authors.forename: Satoru
authors.initials: S
authors.lastname: Joshita
authors.forename: Yasufumi
authors.initials: Y
authors.lastname: Takahashi
authors.forename: Fumihiro
authors.initials: F
authors.lastname: Ishida
authors.forename: Masanori
authors.initials: M
authors.lastname: Matsumoto
authors.forename: Masahito
authors.initials: M
authors.lastname: Uemura
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Fujimura
mesh_terms: D051722:ADAM Proteins / Q000172:deficiency* / Q000276:immunology; D000071120:ADAMTS13 Protein; D005260:Female; D019698:Hepatitis C, Chronic / Q000188:drug therapy / Q000276:immunology / Q000378:metabolism*; D006801:Humans; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000009:adverse effects* / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011092:Polyethylene Glycols / Q000009:adverse effects* / Q000627:therapeutic use; D011697:Purpura, Thrombotic Thrombocytopenic / Q000139:chemically induced* / Q000175:diagnosis / Q000276:immunology; D011994:Recombinant Proteins
chemical_list: D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins; D011092:Polyethylene Glycols; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human; C100416:peginterferon alfa-2a