Treatment of Thrombotic Thrombocytopenic Purpura in Remission with Persistently Low Plasma ADAMTS13 Activity

Achieving clinical remission in immune-mediated TTP does not always mean the underlying immunological risk has resolved. Patients who remain in remission but continue to show low plasma ADAMTS13 activity with no clinical signs or symptoms represent a distinct and important clinical scenario requiring careful management consideration.

Clinical scenario

Immune-mediated TTP (iTTP) in clinical remission with persistently low plasma ADAMTS13 activity, no active signs or symptoms of disease, and not pregnant. Despite the absence of overt clinical relapse, the serological finding of low ADAMTS13 activity indicates continued susceptibility and warrants a proactive approach.

Treatment approach

Current evidence supports a prophylactic intervention in this setting, aimed at reducing relapse risk in patients who would otherwise remain untreated. The specific agent, eligibility criteria, and full clinical guidance are detailed in the complete structured protocol.

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References

DOI: 10.1111/jth.15010

For patients with iTTP who are in remission, but still have low plasma ADAMTS13 activity with no clinical signs/symptoms, the panel suggests the use of rituximab over nonuse of rituximab for prophylaxis.

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