P051: POSTPARTUM HELLP SYNDROME: A RARE OCCURRENCE OF A COAGULOPATHY DISORDER
Andres Bacigalupo Landa, MD, Howard S Goldman, MD, Gerald P Rosen, MD; Mount Sinai Medical Center
INTRODUCTION: HELLP is a complication of pregnancy which refers to the syndrome composed of microangiopathic hemolytic anemia, elevated liver enzymes and low platelet count. Its pathogenesis is unclear: it has been related to preeclampsia, although up to 20% of cases do not have antecedent hypertension or proteinuria. 70% of cases present in the prenatal period, and 30% of the cases are diagnosed postpartum. We describe the case of a primigravid 34-year-old woman with no prior history of hypertension or proteinuria who presented postpartum HELLP syndrome 72 hours after delivery, after having developed disseminated intravascular coagulation (DIC) immediately after delivery.
CASE PRESENTATION: A 34-year-old woman G1P0 with no past medical history presented in labor at 39 weeks of uncomplicated gestation. On arrival, was normotensive and her coagulation panel was within normal limits. An epidural catheter was uneventfully placed during the active phase of labor.
30 minutes after spontaneous vaginal delivery (EBL: 400ml), she was noted to have excessive vaginal bleeding. Upon noticing uterine atony, uterine massage, Misoprostol 800mcg SL and Carbaprost 250mg IM were administered. Because of persistent bleeding, the patient was taken to the OR and, after the epidural anesthetic was optimized, a vaginal exam was performed. Due to persistent bleeding, the decision was made to proceed with an exploratory laparotomy under general anesthesia.
A DIC panel was sent and came back positive. After administering multiple blood products, both uterine arteries were ligated and uterine compression sutures were placed. Total EBL was 1800ml. She was kept intubated and admitted to the SICU.
On POD #1, the patient presented AKI, and HD was started. On POD#2, after the coagulation status was normalized, the patient was extubated.
On POD#3, the patient started complaining of headache, and worsening anemia was noted with no obvious bleeding source. Laboratory work showed microangiopathic hemolytic anemia (peripheral blood smear reported schistocytes), thrombocytopenia and elevated liver enzymes, and the diagnosis of HELLP syndrome was made. She remained hemodynamically stable (normotensive) during this time
40 days after delivery, the patient was discharged home.
DISCUSSION: HELLP syndrome develops in 0.7-0.9% of pregnancies, 70% of the time before delivery, and 80% of patients have history of preeclampsia. This is the fourth case described in the literature of postpartum HELLP syndrome, the 1stcase occurring 72 hours postpartum and with the BP within normal range. This supports the hypothesis that HELLP syndrome may be a separate entity than preeclampsia.
HELLP syndrome has been related to significant maternal mortality (3.9%) and morbidity, with DIC present in 21% of the time. In this case, DIC occurred 3 days prior HELLP syndrome. This raises the question: what if coagulopathy is part of the etiology of HELLP syndrome rather than being a result of it?
In conclusion, HELLP syndrome is a serious complication of pregnancy, mostly presenting before delivery. Postnatal presentation has higher rates of complications, making a prompt diagnosis critical. Its pathophysiology is unclear; it is not always related to preeclampsia, and coagulopathy may be part of the etiology rather than a consequence of it.