血液疾患と輸血ジャーナル

概要

Clinical and Therapeutic Studies of Acquired Thrombotic Thrombocytopenic Purpura in China

Zhaoyue Wang, Ling Sun, Ziqiang Yu, Jian Su, Jing Wang, Haifei Chen and Changgeng Ruan

Thrombotic thrombocytopenic purpura (TTP) is a rare and severe disorder mediated by autoantibodies against ADAMTS13. In this report, we study the clinical features, laboratory aberration, and treatment effect of 55 patients with acquired TTP in China. The classic pentad occurred in only 33% of TTP patients. Severe ADAMTS13 deficiency was detected in 85% of patients. Advanced age and hyperbilirubinemia might be risk factors for poor prognosis. Early and sufficient plasma exchange is the most important approach. The addition of rituximab to plasma exchange and corticosteroids appears to be effective in inducing and sustaining long-term remission in TTP, and is suitable to be administrated during the first episode. However, more optimal therapeutic regimen warrants further investigation to treat refractory cases and to reduce relapse rate.