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HELLP Syndrome
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HELLP syndrome is a group of symptoms that occurs in pregnant women who have pre-eclampsia or eclampsia and who also show signs of liver damage and abnormalities in blood clotting.
It is characterised by:
- Haemolysis
- EL (elevated liver) enzymes
- LP (low platelet) count
HELLP syndrome occurs in 0.5 to 0.9% of all pregnancies and in 10-20% of cases with severe pre-eclampsia.1
- It may occur well before term.2
- HELLP may occur after pre-eclampsia is diagnosed (the norm) or be the first warning of pre-eclampsia when misdiagnosed as, for example, hepatitis or thrombotic thrombocytopenic purpura (TTP).
- It may occur in association with antiphospholipid syndrome.3
- Smoking in pregnancy has been found to cause an 80% reduction in the risk of developing HELLP.4
80% of women with HELLP present before term.
Symptoms
Progressive nausea and vomiting
Upper abdominal pain
Headache
Vision problems
Signs
Jaundice
Upper abdominal tenderness, especially in the right upper quadrant
Hepatomegaly
Easy bruising/purpura
Liver function tests - AST, ALT and LDH elevated.
FBC - Haemoglobin and platelet count reduced.
- Acute fatty liver of pregnancy
- TTP6
- Haemolytic uraemic syndrome
- Acute exacerbation of systemic lupus erythematosus
The main treatment is to deliver the baby as soon as possible, even if premature, since liver function in the mother gets worse very quickly. Problems with the liver can be harmful to both mother and child.
Pharmacological
- Dexamethasone - in gestational ages between 24 and 34 weeks most authors prefer a single course of corticosteroid therapy for fetal lung maturation, either 2 doses of 12 mg betamethasone 24 hours apart or 6 mg of dexamethasone 12 hours apart before delivery. Standard corticosteroid treatment is of uncertain clinical value in the maternal HELLP syndrome. High-dose treatment and repeated doses should be avoided for fear of long-term adverse effects on the fetal brain.1
- Plasma exchange therapy has been successfully used in selected patients with HELLP, who have organ failure or who are refractory to treatment.
Surgery
Women with severe liver damage may need liver transplantation.
- If HELLP is not treated early, up to 25% of women may develop serious complications.
- Without treatment there is a significant mortality.
- The mortality rate among babies born to mothers with HELLP syndrome varies and depends mainly on gestation and birth weight.
- The maternal liver may haemorrhage or rupture.7
- Permanent liver damage or necrosis,8 which may need transplantation, may occur if delivery is delayed.
- Intraventricular haemorrhage with subsequent hydrocephalus has been reported.9
- Retinal detachment and other eye problems have been reported.10
- Transient diabetes insipidus may follow HELLP syndrome.11
Document references
- Haram K, Svendsen E, Abildgaard U; The HELLP syndrome: clinical issues and management. A Review. BMC Pregnancy Childbirth. 2009 Feb 26;9:8. [abstract]
- Haram K, Trovik J, Sandset PM, et al; Severe syndrome of hemolysis, elevated liver enzymes and low platelets (HELLP) in the 18th week of pregnancy associated with the antiphospholipid-antibody syndrome. Acta Obstet Gynecol Scand. 2003 Jul;82(7):679-80.
- Le Thi Thuong D, Tieulie N, Costedoat N, et al; The HELLP syndrome in the antiphospholipid syndrome: retrospective study of 16 cases in 15 women. Ann Rheum Dis. 2005 Feb;64(2):273-8. [abstract]
- Leeners B, Neumaier-Wagner P, Kuse S, et al; Smoking and the risk of developing hypertensive diseases in pregnancy: what is the effect on HELLP syndrome? Acta Obstet Gynecol Scand. 2006;85(10):1217-24. [abstract]
- Sibai BM; Imitators of severe preeclampsia. Obstet Gynecol. 2007 Apr;109(4):956-66. [abstract]
- Rehberg JF, Briery CM, Hudson WT, et al; Thrombotic thrombocytopenic purpura masquerading as hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome in late pregnancy. Obstet Gynecol. 2006 Sep;108(3 Pt 2):817-20. [abstract]
- Mallick IH, Syed SA, Kar AK; Liver rupture following delivery: HELLP needed. Emerg Med J. 2007 May;24(5):372.
- O'Brien J, Buckley O, Munk PL, et al; An unusual case of elevated liver enzymes (2006: 10b). Hepatic necrosis following HELLP syndrome. Eur Radiol. 2007 Jan;17(1):289-91. Epub 2006 Dec 19. [abstract]
- Hirashima C, Ohkuchi A, Matsubara S, et al; Hydrocephalus after intraventricular hemorrhage in eclamptic woman with HELLP syndrome. Hypertens Pregnancy. 2006;25(3):255-7. [abstract]
- Tranos PG, Wickremasinghe SS, Hundal KS, et al; Bilateral serous retinal detachment as a complication of HELLP syndrome. Eye. 2002 Jul;16(4):491-2.
- Ellidokuz E, Uslan I, Demir S, et al; Transient postpartum diabetes insipidus associated with HELLP syndrome. J Obstet Gynaecol Res. 2006 Dec;32(6):602-4. [abstract]
Internet and further reading
- Jung DC, Erogul M; Pregnancy, Pre-eclampsia. eMedicine, May 2008.
Document ID: 7096
Document Version: 2
Document Reference: bgp26101
Last Updated: 5 Oct 2009
Planned Review: 5 Oct 2011
The authors and editors of this article are employed to create accurate and up to date content reflecting reliable research evidence, guidance and best clinical practice. They are free from any commercial conflicts of interest. Find out more about updating.
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