The Japanese experience with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome☆
Abstract
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 responders to plasma exchange, but others were low responders to plasma exchange. The former patients were associated with “idiopathic” TTP, drugs, and pregnancy, and the latter patients with malignancy and stem cell transplantation. Patients with autoimmune disease-associated TTP fit into both groups.
aDepartment of Blood Transfusion Medicine, Nara Medical University, Nara, Japan
bDepartment of 2nd Internal Medicine, Nara Medical University, Nara, Japan
cDepartment of Health Science, Nara Medical University, Nara, Japan
dDepartment of Clinical Laboratory, Mie University School of Medicine, Tsu, Japan
Address correspondence to Yoshihiro Fujimura, MD, Shijyo-cho 840, Kashihara City, Nara 634-8522, Japan
☆ Supported in part by Grants-in-Aid for Scientific Research from the Japanese Ministry of Education, Culture, Sports, Science and Technology (to H.I. and Y.F.) and for Blood Coagulation Abnormalities from the Ministry of Health and Welfare of Japan (to Y.F.).
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