Le HELLP syndrome dans tous ses états

Abstract

AbstractThe HELLP syndrome, first described in 1982, is based on a biological triad involving biological hemolysis, cytolysis, and peripheral thrombocytopenia. HELLP often complicates preeclampsia and typically manifests toward the end of pregnancy or in the postpartum period. Its pathophysiology stems from a defective trophoblastic implantation, leading to placental ischemia and endothelial lesions. Recent publications highlight the likely involvement of the complement system in the pathophysiology of this condition, mimicking the clinical presentation of a non-placental thrombotic microangiopathy (similar to TTP and aHUS). This could partly explain the incidence of renal failure, which is not uncommon in this disease (30 % of cases) and often leads to diagnostic errors. Some authors even advocate for discussing this as a complement-mediated or non-complement-mediated disease, suggesting that in certain situations, there might be an overactivation of the alternate pathway. However, to date, there is no rapid complement activation test available, and the diagnosis relies on clusters of biological evidence lacking sensitivity and specificity. Exploring this line of thinking could potentially broaden the therapeutic options for this condition, which currently rely solely on pregnancy termination.