| Korean expert consensus on the management of thrombotic thrombocytopenic purpura |
Seo-Yeon Ahn1, Junshik Hong2, Jin Seok Kim3, Sung-Hyun Kim4, Kyoung Ha Kim5, Ho-Young Yhim6, Yoo Jin Lee7, Jaewoo Song8, Soo-Mee Bang9, Ji Hyun Lee4, Seongsoo Jang10, Sung Hwa Bae11
, and on behalf of the Korean Society of Hematology Thrombosis and Hemostasis Working Party and the Korean Society on Thrombosis and Hemostasis |
1Department of Hematology-Oncology, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Hwasun, Korea
2Division of Hematology-Medical Oncology, Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
3Division of Hematology, Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
4Division of Hematology and Oncology, Department of Internal Medicine, Dong-A University Hospital, Dong-A University College of Medicine, Busan, Korea
5Department of Oncology and Hematology, Soonchunhyang University Seoul Hospital, Soonchunhyang University College of Medicine, Seoul, Korea
6Department of Internal Medicine, Jeonbuk National University Hospital, Jeonbuk National University Medical School, Jeonju, Korea
7Department of Hematology and Oncology, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea
8Department of Laboratory Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
9Division of Hematology-Medical Oncology, Department of Internal Medicine, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea
10Department of Laboratory Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
11Division of Hematology and Oncology, Department of Internal Medicine, Daegu Catholic University Hospital, Daegu Catholic University School of Medicine, Daegu, Korea |
Corresponding Author:
Sung Hwa Bae , Tel: +82-53-650-4388, Fax: +82-53-622-6062, Email: sunghwa@cu.ac.kr
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Received: October 17, 2025; Revised: December 19, 2025; Accepted: January 17, 2026. |
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| Abstract |
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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. |
| Keywords:
Thrombotic thrombocytopenic purpura ; Diagnosis ; Therapy ; Consensus ; Korea |
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