The Japanese experience with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome
M Matsumoto, H Yagi, H Ishizashi, H Wada… - Seminars in …, 2004 - Elsevier
A total of 290 Japanese patients with thrombotic thrombocytopenic purpura-hemolytic
uremic syndrome (TTP-HUS) were analyzed with respect to ADAMTS-13 activity and its
inhibitor. Twenty-one patients (17 families) had Upshaw-Schulman syndrome, and 12
patients (six families) had familial HUS of undetermined etiology. The number of patients
with acquired HUS and TTP was 44 and 213, respectively. In acquired TTP, patients with
severe deficiency of ADAMTS-13 activity secondary to the presence of an inhibitor were high …
uremic syndrome (TTP-HUS) were analyzed with respect to ADAMTS-13 activity and its
inhibitor. Twenty-one patients (17 families) had Upshaw-Schulman syndrome, and 12
patients (six families) had familial HUS of undetermined etiology. The number of patients
with acquired HUS and TTP was 44 and 213, respectively. In acquired TTP, patients with
severe deficiency of ADAMTS-13 activity secondary to the presence of an inhibitor were high …