title: Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in a population from Japan.
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 in the other cohort). We conclude that in one of the largest cohorts of ai-TTP with severe deficiency of ADAMTS13 activity reported to date, demographic characteristics differ in Japanese patients relative to those reported from a large Caucasian registry from Western societies. Additional studies exploring these findings are needed.
authors.affiliation: Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Japan.
authors.forename: Masanori
authors.initials: M
authors.lastname: Matsumoto
authors.forename: Charles L
authors.initials: CL
authors.lastname: Bennett
authors.forename: Ayami
authors.initials: A
authors.lastname: Isonishi
authors.forename: Zaina
authors.initials: Z
authors.lastname: Qureshi
authors.forename: Yuji
authors.initials: Y
authors.lastname: Hori
authors.forename: Masaki
authors.initials: M
authors.lastname: Hayakawa
authors.forename: Yoko
authors.initials: Y
authors.lastname: Yoshida
authors.forename: Hideo
authors.initials: H
authors.lastname: Yagi
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Fujimura
mesh_terms: D051722:ADAM Proteins / Q000097:blood / Q000172:deficiency*; D000071120:ADAMTS13 Protein; D000367:Age Factors; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000453:epidemiology* / Q000378:metabolism / Q000473:pathology; D018709:Statistics, Nonparametric
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human