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Recombinant ADAMTS-13 for congenital and immune thrombotic thrombocytopenic purpura in pregnancy
Lara Howells1, Maxine Lissack2, Clare Wykes3
1Department of Haematology, University College London Hospitals, London, United Kingdom.
Insights
Recombinant ADAMTS-13 (rADAMTS-13) offers a novel treatment for thrombotic thrombocytopenic purpura (TTP) during pregnancy. This approach achieved excellent outcomes in congenital and immune-mediated TTP cases, improving fetomaternal health.
Area of Science:
- Hematology
- Reproductive Medicine
- Genetics
Background:
- Thrombotic thrombocytopenic purpura (TTP) is a critical condition.
- Congenital TTP (cTTP) and immune-mediated TTP (iTTP) stem from ADAMTS-13 enzyme issues.
- Pregnancy can exacerbate both cTTP and iTTP.
Purpose of the Study:
- To evaluate recombinant ADAMTS-13 (rADAMTS-13) in managing TTP during pregnancy.
- To present case studies of TTP treatment in pregnant individuals using rADAMTS-13.
Main Methods:
- Case series involving three pregnant women with TTP.
- Treatment with rADAMTS-13 for congenital TTP throughout pregnancy.
- rADAMTS-13 used as an alternative to plasma exchange for refractory immune-mediated TTP.
Main Results:
- Two pregnancies with cTTP successfully managed with rADAMTS-13.
- One case of refractory iTTP treated effectively with rADAMTS-13, replacing plasma exchange.
- All three pregnancies achieved excellent fetomaternal outcomes.
Conclusions:
- Recombinant ADAMTS-13 (rADAMTS-13) is a viable and effective therapeutic option for TTP in pregnancy.
- rADAMTS-13 demonstrates promise in improving outcomes for both congenital and immune-mediated TTP during gestation.
- This study highlights a significant advancement in managing TTP during pregnancy, ensuring positive maternal and fetal health.
Abstract:
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening emergency. Congenital TTP (cTTP) and immune-mediated TTP (iTTP) are caused by an inherited severe deficiency of, or immune-mediated destruction of the ADAMTS-13 enzyme, respectively. Pregnancy is recognized as a trigger for both subtypes of this condition. Until recently, the treatment for TTP in pregnancy has involved therapeutic plasma exchange and immunosuppression or plasma infusion. Here, we report 3 cases of TTP in pregnancy: 2 women with cTTP, treated throughout pregnancy with recombinant ADAMTS-13 (rADAMTS-13), and 1 with refractory iTTP, where rADAMTS-13 was used in place of ongoing plasma exchange. All pregnancies resulted in excellent fetomaternal outcomes.
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