Recombinant ADAMTS-13 for congenital and immune thrombotic thrombocytopenic purpura in pregnancy

Blood Vessels Thrombosis & Hemostasis · Published 2025-08-04 · DOI 10.1016/j.bvth.2025.100101

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Abstract

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.

Abstract from DOAJ. Public domain (CC0 1.0).

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Publication details

Year
2025

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