title: Von Willebrand factor-cleaving protease activity remains at the intermediate level in thrombotic thrombocytopenic purpura.
abstract: A 59-year-old woman, diagnosed with chronic myelogenous leukemia (chronic phase) and treated with interferon-alpha for 13 years, developed renal failure. Renal biopsy showed thrombotic thrombocytopenic purpura, but intensive therapy including plasma exchange and steroid administration was not effective. The activity of von Willebrand factor-cleaving protease was detectable at the intermediate level (15-46%) during the clinical course, suggesting that this case was not compatible with the previously reported pattern of idiopathic or drug-induced thrombotic thrombocytopenic purpura, but with the pattern associated with malignant disease or immunological disorders. Further studies to determine the effects of interferon-alpha on endothelial cells in chronic myelogenous leukemia patients are needed.
authors.affiliation: Hematology Oncology Division, Department of Medicine, Hyogo Medical Center for Adults, Akashi, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Sugimoto
authors.forename: Katsuyasu
authors.initials: K
authors.lastname: Saigo
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Shin
authors.forename: Yohji
authors.initials: Y
authors.lastname: Kaneda
authors.forename: Nobuya
authors.initials: N
authors.lastname: Manabe
authors.forename: Hiroko
authors.initials: H
authors.lastname: Narita
authors.forename: Jun
authors.initials: J
authors.lastname: Wakuya
authors.forename: Shion
authors.initials: S
authors.lastname: Imoto
authors.forename: Tohru
authors.initials: T
authors.lastname: Murayama
authors.forename: Masanori
authors.initials: M
authors.lastname: Matsumoto
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Fujimura
authors.forename: Ryuichiro
authors.initials: R
authors.lastname: Nishimura
authors.forename: Tamio
authors.initials: T
authors.lastname: Koizumi
authors.forename: Shunichi
authors.initials: S
authors.lastname: Kumagai
mesh_terms: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D018450:Disease Progression; D005260:Female; D006801:Humans; D016898:Interferon-alpha / Q000008:administration & dosage; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000150:complications / Q000188:drug therapy / Q000201:enzymology*; D008666:Metalloendopeptidases / Q000097:blood*; D008875:Middle Aged; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000201:enzymology* / Q000209:etiology / Q000628:therapy; D051437:Renal Insufficiency / Q000209:etiology / Q000378:metabolism; D013256:Steroids / Q000627:therapeutic use
chemical_list: D016898:Interferon-alpha; D013256:Steroids; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human