Is the HELLP syndrome due to inherited factors? Report of two cases

Servizio di Immunoematologia e Trasfusione, Ospedale Civile di Alessandria, Italy.

The etiology and pathogenesis of the HELLP syndrome, a multisystem disease occurring only in pregnancy, are still unclear. Curiously, very few authors have investigated whether inherited factors may be involved. We report two cases of HELLP syndrome in two unrelated women whose fetuses were relatives (first cousins). The first case concerned a woman aged 32 with a normal course pregnancy who was admitted to the hospital for fever, nausea and vomiting, low platelets, hemolysis and increased liver enzymes. Abruptio placentae with fetal death and severe disseminated intravascular coagulation with hemorrhages ensued within a few hours. Hysterectomy was then performed. After treatment with transfusions and drugs the patient slowly improved; 28 days later she left the hospital in good condition. The second case involved a woman aged 31 with a normal course pregnancy who was admitted to the hospital for epigastric pain, nausea, low platelets, hemolysis and increased liver enzymes. The patient underwent an immediate cesarean section and delivered a live infant; no bleeding occurred during or after delivery. The patient's condition rapidly improved and she left the hospital after 13 days. Until now, no author has proved that inherited fetal factors are at work in the HELLP syndrome. Our observations suggest a role for genetic factors, and this needs to be investigated in prospective studies.