Von Willebrand Factor-Cleaving Protease Activity Remains at the Intermediate Level in Thrombotic Thrombocytopenic Purpura

Publisher： Karger

E-ISSN： 1421-9662|113|3|198-203

ISSN： 0001-5792

Source： Acta Haematologica, Vol.113, Iss.3, 2005-05, pp. : 198-203

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Abstract

A 59-year-old woman, diagnosed with chronic myelogenous leukemia (chronic phase) and treated with interferon-α 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-α on endothelial cells in chronic myelogenous leukemia patients are needed.