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Blood, 15 September 2001, Vol. 98, No. 6, pp. 1842-1846
HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY
Decreased von Willebrand factor protease activity associated with
thrombocytopenic disorders
Jane C. Moore,
Catherine P. M. Hayward,
Theodore E. Warkentin, and
John G. Kelton
From the Department of Medicine, Faculty of Health
Science, McMaster University, Hamilton, ON, Canada.
Recent studies investigating thrombotic thrombocytopenic purpura
(TTP) have implicated abnormal plasma von Willebrand factor (vWF)-cleaving metalloprotease activity in this disorder. It has been
proposed that a metalloprotease cleaves unusually large (UL) multimers
of vWF, which enter the circulation from the endothelium. Abnormal
metalloprotease activity could result in ULvWF, which could participate
in TTP. However, the diagnostic specificity of abnormalities in the
plasma metalloprotease activity has not been established. A prospective
study of vWF protease activity was performed using samples from 20 healthy controls, 20 patients with acute TTP, 20 patients with immune
idiopathic thrombocytopenic purpura (ITP), 10 patients with
disseminated intravascular thrombocytopenia (DIC), 10 patients with
systemic lupus erythematosus (SLE,) and 5 thrombocytopenic patients
with leukemia. Studies were performed blinded to the diagnosis. Samples
from hospitalized patients with normal platelet counts were also
tested. The vWF digests and multimer analysis were done using
previously described methods. Six laboratory personnel independently
scored each of the multimer gels. Reduced protease activity was
observed in 9 of 20 patients with TTP. Reduced activity was also
observed in 6 of 20 patients with ITP, 6 of 10 patients with DIC, 5 of
10 patients with SLE, 1 of 5 patients with leukemia, 2 of 20 healthy
controls, and 3 of 25 hospitalized patients. This study indicates that
abnormalities of vWF protease activity are not restricted to patients
with the diagnosis of TTP.
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