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

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.