Korean expert consensus on the management of thrombotic thrombocytopenic purpura.

Authors

Seo-Yeon Ahn, Junshik Hong, Jin Seok Kim, Sung-Hyun Kim, Kyoung Ha Kim, Ho-Young Yhim, Yoo Jin Lee, Jaewoo Song, Soo-Mee Bang, Ji Hyun Lee, Seongsoo Jang, Sung Hwa Bae, Korean Society of Hematology Thrombosis and Hemostasis Working Party and the Korean Society on Thrombosis and Hemostasis

Published in

The Korean journal of internal medicine. Volume 41. Issue 4. Pages 572-584. Epub Jul 01, 2026.

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare but life-threatening thrombotic microangiopathy resulting from severe ADAMTS13 deficiency. Although effective treatments, such as therapeutic plasma exchange, immunosuppressive therapy with rituximab, and caplacizumab, have significantly improved survival, important challenges remain. These include limited diagnostic capacity, barriers to the early use of novel therapies, and long-term complications. In Korea, additional difficulties persist due to the restricted availability of ADAMTS13 antibody testing, reimbursement limitations for rituximab, and the absence of caplacizumab in clinical practice. To address these issues, the Korean Society of Hematology Thrombosis and Hemostasis Working Party convened an expert panel to develop consensus recommendations for the diagnosis and management of TTP. The panel reviewed current international guidelines, pivotal clinical studies, and real-world experiences and adapted them to the Korean clinical setting. This consensus statement provides updated recommendations for diagnostic approaches, initial and adjunctive therapies, management of refractory disease, ADAMTS13 monitoring, and long-term follow-up. By integrating international evidence with local circumstances, this document aims to provide Korean clinicians with practical, upto-date guidance to enhance the routine care of patients with TTP.

PMID:
42438901
Bibliographic data and abstract were imported from PubMed on 13 Jul 2026.

Read full publication at:
Please sign in to see all details.

Stats

1-terrible, 9-excellent. How would you rate this publication? Sign in in to submit your rating.

Post a comment

You need to be signed in to post comments. You can sign in here.

Comments

There are no comments yet.