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

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Abstract

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 and Affiliations

  1. Department of Internal Medicine, School of Medicine, CHA University, Seongnam, Korea

    Moon Ju Jang, So Young Chong & Doyeun Oh

  2. Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea

    In-Ho Kim & Jee-Hyun Kim

  3. Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea

    Chul-Won Jung

  4. Department of Laboratory Medicine, College of Medicine, Catholic University of Korea, Seoul, Korea

    Ja Young Kim

  5. Department of Internal Medicine, College of Medicine, Catholic University of Korea, Seoul, Korea

    Ji-Chan Park

  6. Department of Internal Medicine, Busan National University College of Medicine, Pusan, Korea

    Sun Min Lee

  7. Department of Internal Medicine, Chonnam National University College of Medicine, Gwangju, Korea

    Yeo-Kyeoung Kim

  8. Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea

    Ji-Eun Lee

  9. Department of Laboratory Medicine, University of Ulsan College of Medicine, Seoul, Korea

    Sung-Su Jang

  10. Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea

    Jin-Seok Kim

  11. Department of Internal Medicine, Chungnam National University College of Medicine, Daejeon, Korea

    Deog-Yeon Jo

  12. Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Chuncheon, Korea

    Dae-Young Zang

  13. Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea

    Young-Yiul Lee

  14. Department of Internal Medicine, Chonbuk National University Medical School, Jeonju, Korea

    Ho-Young Yhim

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  1. Moon Ju Jang
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  2. So Young Chong
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  3. In-Ho Kim
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  7. Ji-Chan Park
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  8. Sun Min Lee
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  9. Yeo-Kyeoung Kim
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  12. Jin-Seok Kim
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  14. Dae-Young Zang
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  15. Young-Yiul Lee
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  16. Ho-Young Yhim
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  17. Doyeun Oh
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Corresponding author

Correspondence to Doyeun Oh.

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Jang, M.J., Chong, S.Y., Kim, IH. et al. Clinical features of severe acquired ADAMTS13 deficiency in thrombotic thrombocytopenic purpura: the Korean TTP registry experience. Int J Hematol 93, 163–169 (2011). https://doi.org/10.1007/s12185-011-0771-5

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  • DOI: https://doi.org/10.1007/s12185-011-0771-5

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