International Journal of Hematology

, Volume 93, Issue 2, pp 163–169 | Cite as

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

  • 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
Original Article

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.

Keywords

ADAMTS-13 Thrombotic thrombocytopenic purpura (TTP) Von Willebrand factor (VWF) cleaving protease Plasma exchange (PE) Microangiopathic hemolytic anemia 

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Copyright information

© The Japanese Society of Hematology 2011

Authors and Affiliations

  • Moon Ju Jang
    • 1
  • So Young Chong
    • 1
  • In-Ho Kim
    • 2
  • Jee-Hyun Kim
    • 2
  • Chul-Won Jung
    • 3
  • Ja Young Kim
    • 4
  • Ji-Chan Park
    • 5
  • Sun Min Lee
    • 6
  • Yeo-Kyeoung Kim
    • 7
  • Ji-Eun Lee
    • 8
  • Sung-Su Jang
    • 9
  • Jin-Seok Kim
    • 10
  • Deog-Yeon Jo
    • 11
  • Dae-Young Zang
    • 12
  • Young-Yiul Lee
    • 13
  • Ho-Young Yhim
    • 14
  • Doyeun Oh
    • 1
  1. 1.Department of Internal Medicine, School of MedicineCHA UniversitySeongnamKorea
  2. 2.Department of Internal MedicineSeoul National University College of MedicineSeoulKorea
  3. 3.Department of Internal Medicine, Samsung Medical CenterSungkyunkwan University School of MedicineSeoulKorea
  4. 4.Department of Laboratory Medicine, College of MedicineCatholic University of KoreaSeoulKorea
  5. 5.Department of Internal Medicine, College of MedicineCatholic University of KoreaSeoulKorea
  6. 6.Department of Internal MedicineBusan National University College of MedicinePusanKorea
  7. 7.Department of Internal MedicineChonnam National University College of MedicineGwangjuKorea
  8. 8.Department of Internal MedicineKorea University College of MedicineSeoulKorea
  9. 9.Department of Laboratory MedicineUniversity of Ulsan College of MedicineSeoulKorea
  10. 10.Department of Internal MedicineYonsei University College of MedicineSeoulKorea
  11. 11.Department of Internal MedicineChungnam National University College of MedicineDaejeonKorea
  12. 12.Department of Internal MedicineHallym University Sacred Heart Hospital, Hallym University College of MedicineChuncheonKorea
  13. 13.Department of Internal MedicineHanyang University College of MedicineSeoulKorea
  14. 14.Department of Internal MedicineChonbuk National University Medical SchoolJeonjuKorea

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