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2026-07-13 PubMed

Korean Expert Panel Develops Comprehensive Consensus Guidelines for Thrombotic Thrombocytopenic Purpura (TTP) Management

Korean expert consensus on the management of thrombotic thrombocytopenic purpura.

Background

Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy primarily caused by severe ADAMTS13 deficiency. While treatments like therapeutic plasma exchange, immunosuppression with rituximab, and caplacizumab have improved survival, significant challenges persist. In Korea, these are compounded by restricted ADAMTS13 antibody testing, rituximab reimbursement limitations, and the absence of caplacizumab. This creates a critical gap for clinicians needing localized, practical guidance.

Key Findings

  • Developed updated recommendations for TTP diagnostic approaches.
  • Provided guidance on initial and adjunctive therapies for TTP management.
  • Addressed management strategies for refractory TTP disease.
  • Included protocols for ADAMTS13 monitoring and long-term follow-up.

korean-consensus ttp thrombotic-thrombocytopenic-purpura adamts13-deficiency hematology plasma-exchange
Source: pubmed:42438901 · Ingested 2026-07-13 · Digest: gemini-2.5-flash