HELLP syndrome--a pregnancy disorder with poor prognosis

HELLP syndrome is a pregnancy-specific disorder defined by hemolysis, elevated liver enzymes and low platelet count that is found in parturients, more frequent in older multiparas. It is frequently associated with severe preeclampsia or eclampsia, but can also be diagnosed in the absence of these disorders. The etiology of HELLP syndrome is unknown, and the pathogenesis of this disorder (including the hepatological manifestations) is not fully understood. The most widely accepted hypotheses are: a change in the immune feto-maternal balance, platelet aggregation, endothelial dysfunction, arterial hypertension and an inborn error of the fatty acid oxidative metabolism. Hepatic involvement occurs by intravascular fibrin deposition and hypovolemia. Serum LDH and platelet count are the two most important clinical tools for disease assessment. LDH reflects both the extent of hemolysis and hepatic dysfunction. Maternofetal complications cause a 7.0-70.0% perinatal mortality rate and a 1.0-24.0% maternal mortality rate. The recognition of HELLP syndrome and an aggressive multidisciplinary approach and prompt transfer of these women to obstetric centers with expertise in this field are required for the improvement of materno-fetal prognosis.