Hyperreactio Luteinalis: Possible Cause of Intrahepatic Cholestasis and Disseminated Intravascular Coagulation [22B

Abstract

INTRODUCTION: Hyperreactio luteinalis (HL) is a rare finding in pregnancy consisting of enlarged multicystic ovaries capable of producing excess human chorionic gonadotropin (hCG) and androgens. We present a patient with HL and twin gestation complicated by intrahepatic cholestasis of pregnancy (ICP) who presented to our institution with disseminated intravascular coagulation (DIC). METHODS: Case Report. RESULTS: A 31-year-old gravida 1 para 0 at 35 weeks 5 days gestation with dichorionic/diamniotic twins, presented to our institution for evaluation due to elevated liver enzymes. On presentation, the patient was jaundiced but asymptomatic with normal vital signs. Fetal assessment revealed category three fetal heart tracing for both fetuses. The patient underwent emergent cesarean delivery. During surgery, bilateral enlarged multicystic ovaries were noted and lab values were consistent with disseminated intravascular coagulation. The patient was treated with fresh frozen plasma and cryoprecipitate then transferred to the intensive care unit. Postoperatively, the patient was noted to have elevated bile acids, bilirubin, androstenedione and aldosterone. CONCLUSION: Studies demonstrate that estrogens play a role in development of ICP and can impair bile acid secretion. HL produces high levels of androgens that are converted to estrogens by precursors from the fetal adrenal gland and placental aromatase. We propose estrogens produced by HL lead to the development of ICP. Excess estrogen production throughout pregnancy may lead to chronic bile acid accumulation and contribute to development of DIC.