Von Willebrand factor-cleaving protease activity remains at the intermediate level in thrombotic thrombocytopenic purpura.
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.
Sugimoto
Takeshi
T
Hematology Oncology Division, Department of Medicine, Hyogo Medical Center for Adults, Akashi, Japan.
Saigo
Katsuyasu
K
Shin
Tomohiro
T
Kaneda
Yohji
Y
Manabe
Nobuya
N
Narita
Hiroko
H
Wakuya
Jun
J
Imoto
Shion
S
Murayama
Tohru
T
Matsumoto
Masanori
M
Fujimura
Yoshihiro
Y
Nishimura
Ryuichiro
R
Koizumi
Tamio
T
Kumagai
Shunichi
S
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
D016898:Interferon-alpha; D013256:Steroids; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human