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

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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.

Original languageEnglish
Pages (from-to)163-169
Number of pages7
JournalInternational Journal of Hematology
Volume93
Issue number2
DOIs
Publication statusPublished - 2011 Feb 1

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All Science Journal Classification (ASJC) codes

  • Hematology

Cite this

Jang, M. J., Chong, S. Y., Kim, I. H., Kim, J. H., Jung, C. W., Kim, J. Y., Park, J. C., Lee, S. M., Kim, Y. K., Lee, J. E., Jang, S. S., Kim, J. S., Jo, D. Y., Zang, D. Y., Lee, Y. Y., Yhim, H. Y., & Oh, D. (2011). Clinical features of severe acquired ADAMTS13 deficiency in thrombotic thrombocytopenic purpura: The Korean TTP registry experience. International Journal of Hematology, 93(2), 163-169. https://doi.org/10.1007/s12185-011-0771-5