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Clinical features of severe acquired ADAMTS13 deficiency in thrombotic thrombocytopenic purpura: the Korean TTP registry experience.

 Moon Ju Jang  ;  So Young Chong  ;  Doyeun Oh  ;  Ho-Young Yhim  ;  Young-Yiul Lee  ;  Dae-Young Zang  ;  Deog-Yeon Jo  ;  Jin-Seok Kim  ;  Sung-Su Jang  ;  Ji-Eun Lee  ;  Yeo-Kyeoung Kim  ;  Sun Min Lee  ;  Ji-Chan Park  ;  Ja Young Kim  ;  Chul-Won Jung  ;  Jee-Hyun Kim  ;  In-Ho Kim 
 International Journal of Hematology, Vol.93(2) : 163-169, 2011 
Journal Title
 International Journal of Hematology 
Issue Date
The clinical significance of ADAMTS13 activity for response to treatment, mortality rate, recurrence, and prognosis is unclear. Therefore, we investigated the characteristics of severe ADAMTS13 deficiency and evaluated its prognostic features in Thrombotic thrombocytopenic purpura (TTP). The Korean TTP Registry includes 66 patients from 13 teaching hospitals in Korea who received the diagnosis of TTP from January 2005 to December 2008. Blood samples obtained upon admission were sent for ADAMTS13 analysis (multimer analysis by sodium dodecyl sulfate electrophoresis) to a central laboratory along with patient clinical information. Patients with severe ADAMTS13 deficiency had lower serum creatinine levels (P = 0.001) than patients with non-severe ADAMTS13 deficiency. Although severe ADAMTS13 deficiency was associated with better response rate (75 vs. 53%, P = 0.145), remission rate (81 vs. 61%, P = 0.209), and mortality rate (19 vs. 31%, P = 0.508) than non-severe ADAMTS13 deficiency, treatment outcomes did not differ significantly between groups. After adjusting for clinical and laboratory features, multivariate analysis did not reveal any independent risk factors for TTP-associated mortality. Patients with severe ADAMTS13 deficiency had lower serum creatinine levels at presentation, but severe ADAMTS13 activity deficiency at TTP diagnosis does not appear to have prognostic significance.
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1. Journal Papers (연구논문) > 1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실)
Yonsei Authors
김진석(Kim, Jin Seok)
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