acquired idiopathic adamts13 activity deficient thrombotic thrombocytopenic purpura in a population from japan特发性adamts13收购活动缺乏从日本人群中血栓性血小板减少性紫癜.pdfVIP

Acquired Idiopathic ADAMTS13 Activity Deficient Thrombotic Thrombocytopenic Purpura in a Population from Japan 1 2 1 2 1 Masanori Matsumoto , Charles L. Bennett , Ayami Isonishi , Zaina Qureshi , Yuji Hori , Masaki 1 1 1 1 Hayakawa , Yoko Yoshida , Hideo Yagi , Yoshihiro Fujimura * 1 Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Japan, 2 South Carolina Center of Economic Excellence for Medication Safety and Efficacy and the Southern Network on Adverse Reactions (SONAR), South Carolina College of Pharmacy, University of South Carolina, Columbia, South Carolina, United States of America Abstract Thrombotic thrombocytopenic purpura (TTP) is a type of thrombotic microangiopathy (TMA). Studies report that the majority of TTP patients present with a deficiency of ADAMTS13 activity. In a database of TMA patients in Japan identified between 1998 and 2008, 186 patients with first onset of acquired idiopathic (ai) ADAMTS13-deficient TTP (ADAMTS13 activity ,5%) were diagnosed. The median age of onset of TTP in this group of patients was 54 years, 54.8% were female, 75.8% had renal involvement, 79.0% had neurologic symptoms, and 97.8% had detectable inhibitors to ADAMTS13 activity. Younger patients were less likely to present with renal or neurologic dysfunction (p,0.01), while older patients were more likely to die during the TTP hospitalization (p,0.05). Findings from this cohort in Japan differ from those reported previously from the United States, Europe, and Korea with respect to age at onset (two decades younger in the other cohort) and gender composition (60% to 100% female