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

Treatment of Relapsed Immune-Mediated Thrombotic Thrombocytopenic Purpura (iTTP)

Immune-mediated TTP (iTTP) can recur after an initial episode. When a patient with a prior iTTP event presents with relapse, the clinical approach requires a specific, structured protocol distinct from a first episode.

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) — relapse in a patient with a documented previous episode of thrombotic thrombocytopenic purpura.
The structured protocol for this scenario involves a combination regimen built around therapeutic plasma exchange (TPE) together with additional agents — including a targeted therapy administered subcutaneously. The full sequence, agent selection, and supporting details are in the complete protocol.

Full regimen, dosing, and clinical guidance available via the protocol link below.

References

DOI: 10.1111/jth.15010

For patients with iTTP experiencing a relapse, the panel recommends addition of corticosteroids to TPE over TPE alone.

For patients with iTTP experiencing an acute event (first event or relapse), the panel suggests using caplacizumab over not using caplacizumab.

Drug acceptability would likely be reasonable because caplacizumab can be administered subcutaneously in an outpatient or home setting via self-administration.

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