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Pregnancy-related thrombotic microangiopathies: Clues from complement biology

Abstract : Pregnancy is a high-risk period for various types of thrombotic microangiopathies (TMA). The improvement of our understanding of the pathophysiology of TMAs has translated into better management of pregnancy-related TMAs. The two main types of TMA, TTP (thrombotic throm-bocytopenic purpura) and hemolytic uremic syndrome (HUS), can both occur during pregnancy and postpartum. TTP is related in most cases to acquired or congenital deficiency of ADAMTS13; it tends to develop mainly during the second and third trimesters of pregnancy. The treatment of pregnancy-TTP aims to restore a detectable ADAMTS13 activity through plasma therapy, and if needed, to induce or sustain remission, immunosuppressive agents. In contrast, HUS develops mainly in the postpartum period. Accumulating data indicate that pregnancy-HUS is an atypical, i.e., complement-mediated HUS, triggered by pregnancy. Its treatment therefore should include the use of the anti-C5 humanized monoclonal antibody eculizumab. In other TMA-like disorders associated with pregnancy, including HELLP (hemolysis, elevated liver enzymes, low platelets) and pre-eclampsia/eclampsia, complement involvement, and the need for specific anti-complement therapies, is an active area of investigation.
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Fadi Fakhouri. Pregnancy-related thrombotic microangiopathies: Clues from complement biology. Transfusion and Apheresis Science, Elsevier, 2016, 54 (2), pp.199-202. ⟨10.1016/j.transci.2016.04.009⟩. ⟨inserm-02149507⟩



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