Clinical characteristics, treatments, and outcomes of thrombotic thrombocytopenic purpura treated with plasma exchange in Japan: a nationwide inpatient database study

Abstract Background Plasma exchange (PEX) has been the primary treatment for immune-mediated thrombotic thrombocytopenic purpura (iTTP) since 1990s. Daily PEX is recommended in international guidelines, but was only reimbursed up to three times weekly under Japanese national health insurance system until March 2018. This study conducted analyze practice patterns and outcomes patients with TTP Japan. Methods We used Diagnosis Procedure Combination inpatient database, including data from approximately 1,200 acute care hospitals. identified all hospitalized adult who were diagnosed on admission July 2010 2017 received at least one during hospitalization, which we defined as treated PEX. revealed patient characteristics, patterns, outcomes. Results There 1,559 The median age 64 (interquartile range [IQR] 46–74) years. slightly more women (59%) than men enrolled into this study. frequency within 7 days of initial (IQR 2–5) times. total number hospitalization six 3–10), while duration 10 4–22) days. time 4 2–11) Overall, in-hospital mortality 32%. Thirty-three percent (144/437) hospitals reported case period. Conclusions Our demonstrated that clinical Japan considerably varied its standardization warranted.