| Clinical features of severe acquired ADAMTS13 deficiency in thrombotic thrombocytopenic purpura: the Korean TTP registry experience. | |
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MedLine Citation:
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PMID: 21287408 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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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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Authors:
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Moon Ju Jang; So Young Chong; In-Ho Kim; Jee-Hyun Kim; Chul-Won Jung; Ja Young Kim; Ji-Chan Park; Sun Min Lee; Yeo-Kyeoung Kim; Ji-Eun Lee; Sung-Su Jang; Jin-Seok Kim; Deog-Yeon Jo; Dae-Young Zang; Young-Yiul Lee; Ho-Young Yhim; Doyeun Oh |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-2-3 |
Journal Detail:
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Title: International journal of hematology Volume: - ISSN: 1865-3774 ISO Abbreviation: - Publication Date: 2011 Feb |
Date Detail:
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Created Date: 2011-2-2 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9111627 Medline TA: Int J Hematol Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
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Department of Internal Medicine, School of Medicine, CHA University, Seongnam, Korea. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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