Highlights & Basics
- HELLP syndrome is considered to be a severe form of preeclampsia (sometimes called "atypical preeclampsia") characterized by hemolysis (H), also expressed as microangiopathic hemolytic anemia, elevated liver enzymes (EL), and low platelets (LP).
- The condition usually occurs antepartum, between 27 and 37 weeks' gestation; 15% to 30% of cases present initially postpartum. HELLP syndrome poses significant diagnostic and therapeutic challenges because only 80% to 85% of affected people present typically with hypertension and proteinuria.
- HELLP syndrome should be considered in any pregnant person presenting in the second half of gestation or immediately postpartum with significant new-onset epigastric/upper abdominal pain until proven otherwise.
- The disease is associated with progressive and sometimes rapid maternal and fetal deterioration.
- Early detection and aggressive management with a combination of intravenous magnesium sulfate, intravenous dexamethasone, control of blood pressure to prevent or minimize severe systolic hypertension, replacement of blood products, as needed, and timely delivery of the fetus and placenta seem to be the best and safest ways to arrest disease progression and reduce adverse outcomes. Maternal outcomes are improved considerably with this management; perinatal outcome depends predominantly on the gestational age when delivery occurs. A critical step is the early initiation of potent glucocorticoids as soon as the diagnosis of HELLP syndrome is made, so that severe maternal morbidity (stroke, liver hematoma/infarction/rupture, acute pancreatitis) and maternal mortality can be avoided.
Quick Reference
History & Exam
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Definition
Epidemiology
Etiology
Pathophysiology
Citations
Martin JN Jr, Blake PG, Perry KG Jr, et al. The natural history of HELLP syndrome: patterns of disease progression and regression. Am J Obstet Gynecol. 1991 Jun;164(6 Pt 1):1500-9; discussion 1509-13.[Abstract]
Martin JN Jr, Brewer JM, Wallace K, et al. Hellp syndrome and composite major maternal morbidity: importance of Mississippi classification system. J Matern Fetal Neonatal Med. 2013 Aug;26(12):1201-6.[Abstract]
Martin JN Jr, Rinehart BK, May WL, et al. The spectrum of severe preeclampsia: comparative analysis by HELLP (hemolysis, elevated liver enzyme levels, and low platelet count) syndrome classification. Am J Obstet Gynecol. 1999 Jun;180(6 Pt 1):1373-84.[Abstract]
American College of Obstetricians and Gynecologists. ACOG practice bulletin no. 222: gestational hypertension and preeclampsia. Jun 2020 [internet publication].[Full Text]
Sibai BM. Diagnosis and management of gestational hypertension and preeclampsia. Obstet Gynecol. 2003 Jul;102(1):181-92.[Abstract]
Martin JN Jr, Owens MY. Preeclampsia-eclampsia y syndrome de HELLP. In: Romero Arauz JF, Tena Alavez G, Jimenez Solis GA, eds. Preeclampsia - enfermedades hipertensivas del embarazo [in Spanish]. Mexico: McGraw Hill; 2012.
Sibai BL, Ramadan MK, Chari RS, et al. Pregnancies complicated by HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets): subsequent pregnancy outcome and long-term prognosis. Am J Obstet Gynecol. 1995 Jan;172(1 Pt 1):125-9. [Abstract]
Duley L, Meher S, Hunter KE, et al. Antiplatelet agents for preventing pre-eclampsia and its complications. Cochrane Database Syst Rev. 2019 Oct 30;2019(10):CD004659.[Abstract][Full Text]
Martin JN Jr, Owens MY, Keiser SD, et al. Standardized Mississippi Protocol treatment of 190 patients with HELLP syndrome: slowing disease progression and preventing new major maternal morbidity. Hypertens Pregnancy. 2012;31(1):79-90.[Abstract]
Magee LA, Pels A, Helewa M, et al; Canadian Hypertensive Disorders of Pregnancy Working Group. Diagnosis, evaluation, and management of the hypertensive disorders of pregnancy: executive summary. J Obstet Gynaecol Can. 2014 May;36(5):416-41.[Abstract][Full Text]
Martin JN Jr. Milestones in the quest for best management of patients with HELLP syndrome (microangiopathic hemolytic anemia, hepatic dysfunction, thrombocytopenia). Int J Gynaecol Obstet. 2013 Jun;121(3):202-7. [Abstract]
Martin JN Jr, Thigpen BD, Moore RC, et al. Stroke and severe preeclampsia and eclampsia: a paradigm shift focusing on systolic blood pressure. Obstet Gynecol. 2005 Feb;105(2):246-54.[Abstract]
Sibai BM. Diagnosis, controversies, and management of the syndrome of hemolysis, elevated liver enzymes, and low platelet count. Obstet Gynecol. 2004 May;103(5 Pt 1):981-91.[Abstract]
O'Brien JM, Barton JR. Controversies with the diagnosis and management of HELLP syndrome. Clin Obstet Gynecol. 2005 Jun;48(2):460-77.[Abstract]
Sibai BL, Ramadan MK, Chari RS, et al. Pregnancies complicated by HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets): subsequent pregnancy outcome and long-term prognosis. Am J Obstet Gynecol. 1995;172:125-129.[Abstract]
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