The syndrome of haemolysis, elevated liver enzymes and low platelets is a rare condition specific to pregnancy, affecting approximately 5–20% of all pre-eclamptic pregnancies. Described here is a woman in her first pregnancy, who experienced an intrauterine death following a significant hepatic haematoma and capsular rupture, in the absence of classical clinical features suggestive of pre-eclampsia. The events that followed suggested haemolysis, elevated liver enzymes and low platelets syndrome as the likely diagnosis. The patient’s clinical course highlights the difficulties that may be encountered when making decisions about pregnant women with complicated medical and obstetric issues.
WeinsteinL. Syndrome of hemolysis, elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy. Am J Obstet Gynecol1982; 142: 159–167.
2.
MurphyDJStirratGM. Mortality and morbidity associated with early-onset preeclampsia. Hypertens Pregnancy2000; 19: 221–231.
3.
Knight M, Tuffnell D, Kenyon SSJ, et al. (Eds); on behalf of MBRRACE-UK. Saving lives, improving mothers’ care – surveillance of maternal deaths in the UK 2011-13 and lessons learned to inform maternity care from the UK and Ireland Confidential Enquiries into Maternal Deaths and Morbidity 2009-13. Oxford: National Perinatal Epidemiology Unit, University of Oxford, 2015.
4.
Centre for Maternal and Child Enquiries (CMACE). Saving mothers' lives: reviewing maternal deaths to make motherhood safer: 2006–2008. The Eighth Report of the Confidential Enquiries into Maternal Deaths in the United Kingdom. BJOG2011; 118(Suppl 1): 1–203.
WestbrookRHYeomanADJoshiD. Outcomes of severe pregnancy-related liver disease: refining the role of transplantation. Am J Transplant2010; 10: 2520–2526.
7.
Ch'ngCL. Prospective study of liver dysfunction in pregnancy in Southwest Wales. Gut2002; 51: 876–880.
8.
PereiraSPO'DonohueJWendonJ. Maternal and perinatal outcome in severe pregnancy-related liver disease. Hepatology1997; 26: 1258–1262.
9.
DoumiriMElombilaMOudghiriN. Ruptured subcapsular hematoma of the liver complicating acute fatty liver of pregnancy. Pan Afr Med J2014; 19: 38–38.
10.
SchwartzMLLienJM. Spontaneous liver hematoma in pregnancy not clearly associated with preeclampsia: a case presentation and literature review. Am J Obstet Gynecol1997; 176: 1328–1332; discussion 32–33.
11.
DeKoninckPLJLoquetPLeymanP. Spontaneous hepatic rupture in a normotensive monoamniotic twin pregnancy: case report and review of the literature. Gynecol Obstet Invest2010; 70: 69–72.
12.
AbdiSCameronICNakielnyRA. Spontaneous hepatic rupture and maternal death following an uncomplicated pregnancy and delivery. BJOG2001; 108: 431–433.
13.
MatsudaYMaedaTHataeM. Spontaneous rupture of the liver in an uncomplicated pregnancy. J Obstet Gynaecol Res1997; 23: 449–452.
14.
MascarenhasRMathiasJVaradarajanR. Spontaneous hepatic rupture: a report of five cases. HPB (Oxford)2002; 4: 167–170.
15.
SibaiBM. Diagnosis, controversies, and management of the syndrome of hemolysis, elevated liver enzymes, and low platelet count. Obstet Gynecol2004; 103: 981–991.
16.
WeinsteinL. It has been a great ride: the history of HELLP syndrome. Am J Obstet Gynecol2005; 193: 860–863.
17.
National Institute for Health and Clinical Excellence. Hypertension in pregnancy: diagnosis and management. Clinical guideline 107. London: NICE, 2010.
18.
HuJZhangQRenX. Efficacy and safety of acetylcysteine in “non-acetaminophen” acute liver failure: a meta-analysis of prospective clinical trials. Clin Res Hepatol Gastroenterol2015; 39: 594–599.
19.
AuzingerGWendonJ. Intensive care management of acute liver failure. Curr Opin Crit Care2008; 14: 179–188.
