Treatment of iTTP in Pregnancy with Decreased ADAMTS13 Activity

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) during pregnancy poses a distinct clinical challenge when plasma ADAMTS13 activity is significantly reduced — even before any overt symptoms appear.

Clinical Scenario

Pregnant patient with immune-mediated TTP (iTTP) and decreased plasma ADAMTS13 activity (e.g., below 30 U/dL or below 30% of normal), in the absence of clinical signs or symptoms. Evidence indicates that pregnant women with this degree of ADAMTS13 reduction may be at risk for poor clinical outcomes even while currently asymptomatic.

Treatment Approach

The recommended approach is prophylactic treatment during pregnancy, with a plasma-based intervention forming the core of management. The complete regimen — including specific thresholds, sequencing, and monitoring — is detailed in the structured protocol.

Clinical Goal

The primary target is an increase in plasma ADAMTS13 activity, aimed at preventing both maternal and fetal mortality and morbidity.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1111/jth.15010

For patients with iTTP who are pregnant and have decreased plasma ADAMTS13 activity but with no clinical signs/symptoms, the panel recommends prophylactic treatment over no prophylactic treatment.
The panel felt that pregnant women with decrease in plasma ADAMTS13 activity (eg, <30 U/dL or <30% of normal) may have poor clinical outcomes.
For asymptomatic iTTP with low plasma ADAMTS13 activity, the panel made a conditional recommendation for the use of rituximab outside of pregnancy, but prophylactic TPE during pregnancy.
Although no specific prophylactic immune suppressive or plasma treatment regimen is recommended, the panel made a strong recommendation for the use of prophylactic treatment to increase the ADAMTS13 activity to prevent both maternal and fetal mortality and morbidity.

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