Document Detail

Registry of 919 patients with thrombotic microangiopathies across Japan: database of Nara Medical University during 1998-2008.
MedLine Citation:
PMID:  20045995     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Thrombotic microangiopathies (TMAs) are pathological conditions characterized by generalized microvascular occlusion by platelet thrombi, thrombocytopenia, and microangiopathic hemolytic anemia. Two typical phenotypes of TMAs are hemolytic-uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). Severe deficiency of plasma ADAMTS13 activity (ADAMTS13: AC) is more specific for TTP, but not for HUS. Since 1998, our laboratory has functioned as a nationwide referral center for TMAs by analyzing ADAMTS13. METHODS: Of 1,564 patients tested from 426 hospitals, 919 were positive for TMA. Levels of ADAMTS13: AC and the ADAMTS13 neutralizing autoantibody (ADAMTS13: INH) were determined by chromogenic act-ELISA and/or by classic von Willebrand factor multimer assay. RESULTS: TMA patients consisted of two groups: severe (less than 3% of normal control) and non-severe deficiency of ADAMTS13: AC. Both groups were divided into congenital (n=65) and acquired (n=854) TMA. Of the former, 41 had congenital deficiency of ADAMTS13: AC, while the remaining 24 had disease of unknown etiology. The 854 patients with acquired TMA could be largely grouped into three categories: idiopathic TTP (n=284), idiopathic HUS (n=106), and secondary TMAs (n=464). The secondary TMAs were observed in heterogeneous patient groups and were associated with drugs, connective tissue diseases, malignancies, transplantation, pregnancy, E. coli O157: H7 infection, and other factors. All of the patients with acquired severe ADAMTS13: AC deficiency were positive for ADAMTS13: INH. CONCLUSION: Although TMAs are highly heterogeneous pathological conditions, one-third of TMA patients have severe deficiency of ADAMTS13: AC. Platelet transfusions to such patients are contraindicated. Rapid ADAMTS13: AC assays are therefore prerequisite to appropriately treat TMA patients.
Yoshihiro Fujimura; Masanori Matsumoto
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-01-01
Journal Detail:
Title:  Internal medicine (Tokyo, Japan)     Volume:  49     ISSN:  1349-7235     ISO Abbreviation:  Intern. Med.     Publication Date:  2010  
Date Detail:
Created Date:  2010-01-04     Completed Date:  2010-04-22     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9204241     Medline TA:  Intern Med     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  7-15     Citation Subset:  IM    
Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Japan.
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MeSH Terms
ADAM Proteins / deficiency*
Hemolytic-Uremic Syndrome / blood,  diagnosis*
Purpura, Thrombotic Thrombocytopenic / blood,  diagnosis*
Reg. No./Substance:
EC 3.4.24.-/ADAM Proteins; EC 3.4.24.-/ADAMTS13 protein, human

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