HELLP Syndrome

OVERVIEW

  • 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

DIAGNOSTIC CRITERIA

Tennessee Classification System diagnostic criteria for HELLP are:

  • haemolysis
  • 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.

PATHOPHYSIOLOGY

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.

CLINICAL FEATURES

History

  • no ‘typical’ clinical symptoms
  • epigastric or RUQ pain
  • weight gain (oedema)

Examination

  • hypertension
  • tender RUQ
  • oedema
  • polyuria from nephrogenic DI

INVESTIGATIONS

  • 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

COMPLICATIONS

Haemorrhage

  • Abruptio placentae
  • Severe postpartum haemorrhage
  • Subcapsular liver haematoma
  • Intracerebral or brainstem haemorrhage
  • DIC

Infarction

  • Liver infarct
  • Cerebral infarct

Pregnancy

  • overlap with pre-eclampsia
  • preterm delivery
  • foetal demise in utero

Other

  • Visual impairment due to retinopathy
  • Pulmonary oedema – higher risk in postpartum onset of HELLP
  • Acute kidney injury – higher risk in postpartum onset of HELLP

DIFFERENTIAL DIAGNOSIS

  • Pre-eclampsia / eclampsia
  • Acute fatty liver of pregnancy
  • Acute hepatitis
  • HUS
  • TTP (rare in pregnancy)
  • ITP
  • DIC (e.g. from PPH or amniotic fluid embolism)
  • other causes of haemolysis (e.g. AIHA, sepsis)
  • other causes of acute abdomen

MANAGEMENT

Resuscitation

  • prepare for major haemorrhage
  • major life threats are hepatic hemorrhage, subcapsular hematoma, liver rupture, and multi-organ failure

Specific treatment

  • 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

Disposition

  • OT or HDU/ ICU setting
  • consider transfer to a liver transplant center

References and  Links

FOAM and web resources

Journal articles

  • 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.

CCC 700 6

Critical Care

Compendium

Chris is an Intensivist and ECMO specialist at the Alfred ICU in Melbourne. He is also the Innovation Lead for the Australian Centre for Health Innovation at Alfred Health and Clinical Adjunct Associate Professor at Monash University. He is a co-founder of the Australia and New Zealand Clinician Educator Network (ANZCEN) and is the Lead for the ANZCEN Clinician Educator Incubator programme. He is on the Board of Directors for the Intensive Care Foundation and is a First Part Examiner for the College of Intensive Care Medicine. He is an internationally recognised Clinician Educator with a passion for helping clinicians learn and for improving the clinical performance of individuals and collectives.

After finishing his medical degree at the University of Auckland, he continued post-graduate training in New Zealand as well as Australia’s Northern Territory, Perth and Melbourne. He has completed fellowship training in both intensive care medicine and emergency medicine, as well as post-graduate training in biochemistry, clinical toxicology, clinical epidemiology, and health professional education.

He is actively involved in in using translational simulation to improve patient care and the design of processes and systems at Alfred Health. He coordinates the Alfred ICU’s education and simulation programmes and runs the unit’s education website, INTENSIVE.  He created the ‘Critically Ill Airway’ course and teaches on numerous courses around the world. He is one of the founders of the FOAM movement (Free Open-Access Medical education) and is co-creator of litfl.com, the RAGE podcast, the Resuscitology course, and the SMACC conference.

His one great achievement is being the father of two amazing children.

On Twitter, he is @precordialthump.

| INTENSIVE | RAGE | Resuscitology | SMACC

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