Thrombotic thrombocytopenic purpura
ICD-10 M31.1 · ICD-11 3B64.14

Treatment of Thrombotic Thrombocytopenic Purpura in Relapsing Immune-Mediated TTP (iTTP)

Clinical Scenario

This protocol covers immune-mediated thrombotic thrombocytopenic purpura (iTTP) in patients who are presenting with a relapse — a recurrent episode following a prior confirmed episode of thrombotic thrombocytopenic purpura.

Specific Situation

The patient carries a history of at least one previous TTP episode and is now experiencing a relapse of the immune-mediated form (iTTP). This relapse context shapes the treatment recommendation and distinguishes it from management at first presentation.

Treatment Approach (Partial Overview)

The evidence-based approach for relapsing iTTP centres on therapeutic plasma exchange (TPE), with a specific additional therapy recommended in combination for the relapse setting. The complete structured protocol — including the full combination, clinical guidance, and sequencing — is available via the link below.

Instant Access to Structured Evidence-Based Regimens
References
DOI: 10.1111/jth.15010
For patients with iTTP experiencing a relapse, the panel recommends addition of corticosteroids to TPE over TPE alone.
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