Thrombotic Microangiopathies Associated with Pregnancy: Diagnostic and Therapeutic Challenges

Several serious disorders may present during pregnancy or postpartum with thrombotic microangiopathy (p-TMA). Signs of microangiopathic hemolytic anemia and thrombocytopenia may arise due to pregnancy complications such as severe preeclampsia (sPE) and HELLP-syndrome (hemolysis, elevated liver enzymes, and low platelets) or severe independent diseases: atypical hemolytic uremic syndrome (aHUS), thrombotic thrombocytopenic purpura (TTP). Recent evidence and clinical similarities suggest a link sPE/HELLP to aHUS, a disease of excessive activation of the alternative complement pathway. Pregnancy-associated aHUS is a severe disorder with a high risk of maternal and fetal morbidity and mortality, defined by the occurrence of comlement-mediated TMA without ADAMTS13 deficiency. Triggered by pregnancy and another complement-amplifying conditions women develop the syndrome, leading to a disastrous hemolytic disease characterized by diffuse endothelial damage and platelet consumption. Delivery is the treatment of choice of sPE and HELLP, but can lead to progression in case of aHUS: even now it is associated with unfavorable outcomes.We observe 116 women with p-TMA: 43 aHUS, 36 HELLP, 35 sPE, 2 TTP. PE diagnosed in accordance with the WHO criteria of 2008. HELLP- in accordance with Tenessee criteria (laboratory parameters normalized beyond 48-72 hours after delivery). TTP was associated with ADAMTS 13 deficiency <10%. We compare results with those of 28 healthy pregnant women. All pa...
Source: Blood - Category: Hematology Authors: Tags: 903. Outcomes Research-Non-Malignant Hematology: Poster III Source Type: research