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Blood, 15 September 2001, Vol. 98, No. 6, pp. 1765-1772
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Specific von Willebrand factor-cleaving
protease in thrombotic microangiopathies: a study of 111 cases
Agnès Veyradier,
Bernadette Obert,
Anne Houllier,
Dominique Meyer, and
Jean-Pierre Girma
From the INSERM Unité 143, Le Kremlin
Bicêtre, France.
Retrospective studies of patients with thrombotic microangiopathies
(TMAs) have shown that a deficient activity of von Willebrand factor
(vWF)-cleaving protease is involved in thrombotic thrombocytopenic purpura (TTP) but not in the hemolytic-uremic syndrome (HUS). To
further analyze the relevance of this enzymatic activity in TMA
diagnosis, a 20-month multicenter study of vWF-cleaving protease activity was conducted in adult patients prospectively enrolled in the
acute phase of TMA. Patients with sporadic (n = 85), intermittent (n = 21), or familial recurrent (n = 5) forms of TMA (66 manifesting as TTP and 45 as HUS) were included. TMA was either
idiopathic (n = 42) or secondary to an identified clinical context
(n = 69). vWF-cleaving protease activity was normal in 46 cases (7 TTP and 39 HUS) and decreased in 65 cases (59 TTP and 6 HUS). A
protease inhibitor was detected in 31 cases and was observed only in
patients manifesting TTP with a total absence of protease activity.
Among the 111 patients, mean vWF antigen levels were increased and the multimeric distribution of vWF was very heterogeneous, showing either a
defect of the high-molecular-weight forms (n = 40), a normal pattern
(n = 21), or the presence of unusually large multimers (n = 50).
Statistical analysis showed that vWF-protease deficiency was associated
with the severity of thrombocytopenia (P < .01). This
study emphasizes that vWF-cleaving protease deficiency specifically concerns a subgroup of TMA corresponding to the TTP entity.
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