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. 2012;7(3):e33029.
doi: 10.1371/journal.pone.0033029. Epub 2012 Mar 12.

Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in a population from Japan

Masanori Matsumoto  1 , Charles L Bennett, Ayami Isonishi, Zaina Qureshi, Yuji Hori, Masaki Hayakawa, Yoko Yoshida, Hideo Yagi, Yoshihiro Fujimura
Affiliations

Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in a population from Japan

Masanori Matsumoto et al. PLoS One. 2012.

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.

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Conflict of interest statement

Competing Interests: The authors have read the journal's policy and have the following conflicts: Y.F. is a member of clinical advisory boards for Baxter BioScience. This does not alter the authors' adherence to all the PLoS ONE policies on sharing data and materials.

Figures

Figure 1
Figure 1. Age distribution and ADAMTS13 inhibitor levels in acquired idiopathic (ai−) TTP with severe deficiency of ADAMTS13 activity.
Upper panel shows the age distribution of 186 patients with severe deficiency of ADAMTS13 activity under 5%. We found wide range of the age at TTP bouts from 8 months old to 87 years old. The highest incident peak was found around 60 years old. Lower panel shows the distribution of ADAMTS13 inhibitors in 186 ai-TTP patients with severe deficiency of ADAMTS13 activity. We found ADAMTS13 inhibitors (≥0.5 BU/ml) in 182 patients (97.8%). High titer inhibitors ≥2.0 BU/ml was seen in 101 patients (54.3%).
See this image and copyright information in PMC

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