- HELLP Syndrome: haemolysis, elevated liver enzymes, and a low platelet count
- there is clear overlap between pre-eclampsia and HELLP syndrome, and it is unclear whether the latter is a primary or secondary disease process.
- typically a third trimester condition, which may occur up to 7 days after delivery
- affects 05-1% pregnancies
- 1-2% mortality
Tennessee Classification System diagnostic criteria for HELLP are:
- increased LDH (> 600 U/L)
- increased AST (>or= 70 U/L)
- low platelets < 100 x 10(9)/L.
The HELLP syndrome may be complete or incomplete.
The Mississippi Triple-class HELLP System further classifies the disorder by the nadir platelet counts.
Generalized endothelial and microvascular injury from
- activation of the complement and coagulation cascades
- increased vascular tone
- platelet aggregation
This results in areas of hemorrhage and necrosis within the liver and may evolve to large haematomas, capsular tears, and intraperitoneal bleeding.
- no ‘typical’ clinical symptoms
- epigastric or RUQ pain
- weight gain (oedema)
- tender RUQ
- polyuria from nephrogenic DI
- microangiopathic haemolytic anaemia (MAHA)
- elevated LFT’s – bilirubin, AST, ALT, LDH
- low platelets
- normal PT, APTT and coag screen
- haemolysis on blood film
- haptoglobins: low
- Abruptio placentae
- Severe postpartum haemorrhage
- Subcapsular liver haematoma
- Intracerebral or brainstem haemorrhage
- Liver infarct
- Cerebral infarct
- overlap with pre-eclampsia
- preterm delivery
- foetal demise in utero
- Visual impairment due to retinopathy
- Pulmonary oedema – higher risk in postpartum onset of HELLP
- Acute kidney injury – higher risk in postpartum onset of HELLP
- Pre-eclampsia / eclampsia
- Acute fatty liver of pregnancy
- Acute hepatitis
- TTP (rare in pregnancy)
- DIC (e.g. from PPH or amniotic fluid embolism)
- other causes of haemolysis (e.g. AIHA, sepsis)
- other causes of acute abdomen
- prepare for major haemorrhage
- major life threats are hepatic hemorrhage, subcapsular hematoma, liver rupture, and multi-organ failure
- Delivery is indicated if the HELLP syndrome occurs after the 34th gestational week or the foetal and/or maternal conditions deteriorate.
- Seek and treat complications (e.g. APO, DIC, MODS)
- anti-hypertensives to keep BP below 155/105 mmHg
— Labetolol or hydralazine or nifedipine
- MgSO4 IV for eclamptic seizure prophylaxis
- corticosteroids (IV)
— no clear benefit for HELLP per se
— given for fetal lung maturity from 24 to 34 weeks: either 2 doses of 12 mg betamethasone 24 hours apart or 6 mg dexamethasone 12 hours apart before delivery.
- Liver haemorrhage
— manage conservatively where possible
— correct coagulopathy
— surgery includes drainage of the hematoma, packing, over-sewing of lacerations, or partial hepatectomy
— consider arterial embolisation
- Exchange transfusion
– considered in situations of progressive elevation of bilirubin or falling Hb or PLTs and ongoing deterioration in maternal condition.
- Novel therapies:
— Antithrombin and glutathione – have been trialed with some benefit demonstrated, but has not yet been subjected to any high quality trial
— Octreotide – no role in HELLP syndrome
— there are case reports of liver transplantation
Supportive care and monitoring
- consider invasive monitoring
- OT or HDU/ ICU setting
- consider transfer to a liver transplant center
References and Links
FOAM and web resources
- Haram K, Svendsen E, Abildgaard U. The HELLP syndrome: clinical issues and management. A Review. BMC Pregnancy Childbirth. 2009 Feb 26;9:8. PMC2654858.
- Lee NM, Brady CW. Liver disease in pregnancy. World J Gastroenterol. 2009 Feb 28;15(8):897-906. PMC2653411.
- McCrae KR. Thrombocytopenia in pregnancy. Hematology Am Soc Hematol Educ Program. 2010;2010:397-402. PMID: 21239825.
- Neligan PJ, Laffey JG. Clinical review: Special populations–critical illness and pregnancy. Crit Care. 2011 Aug 12;15(4):227. PMC3387584.
- Shames BD, Fernandez LA, Sollinger HW, Chin LT, D’Alessandro AM, Knechtle SJ, Lucey MR, Hafez R, Musat AI, Kalayoglu M. Liver transplantation for HELLP syndrome. Liver Transpl. 2005 Feb;11(2):224-8. PMID: 15666378.