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Life in the Fast Lane • LITFL • Medical Blog

Emergency medicine and critical care medical education blog

Critical Care Compendium | HELLP Syndrome

HELLP Syndrome

by Chris Nickson, Last updated May 24, 2016

OVERVIEW

  • 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 hematomas, 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 post partum haemorrhage
  • Subcapsular liver haematoma
  • Intracerebral or brainstem haemorrhage
  • DIC

Infarction

  • Liver infarct
  • Cerebral infarct

Pregnancy

  • overlap with preeclampsia
  • preterm delivery
  • voetal demise in utero

Other

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

DIFFERENTIAL DIAGNOSIS

  • Pre-ecclampsia / 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, oversewing 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 trialled 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

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. doi: 10.1186/1471-2393-9-8. Review. PubMed PMID: 19245695; PubMed Central PMCID: PMC2654858.
  • Lee NM, Brady CW. Liver disease in pregnancy. World J Gastroenterol. 2009 Feb 28;15(8):897-906. Review. PubMed PMID: 19248187; PubMed Central PMCID: PMC2653411.
  • McCrae KR. Thrombocytopenia in pregnancy. Hematology Am Soc Hematol Educ Program. 2010;2010:397-402. doi: 10.1182/asheducation-2010.1.397. Review. PubMed PMID: 21239825. [Free Full Text]
  • Neligan PJ, Laffey JG. Clinical review: Special populations–critical illness and pregnancy. Crit Care. 2011 Aug 12;15(4):227. doi: 10.1186/cc10256. Review. PubMed PMID: 21888683; PubMed Central PMCID: 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. PubMed PMID: 15666378. [Free Full Text]

FOAM and web resources

  • ICN — Hot Case #14 – I’m pregnant … HELLP!

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About Chris Nickson

FCICM FACEM BSc(Hons) BHB MBChB MClinEpid(ClinTox) DipPaeds DTM&H GCertClinSim

Chris is an Intensivist at the Alfred ICU in Melbourne and is an Adjunct Clinical Associate Professor at Monash University. He is also the Innovation Lead for the Australian Centre for Health Innovation and the Chair of the Australian and New Zealand Intensive Care Society (ANZICS) Education Committee. He has 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 since completed further training in emergency medicine, clinical toxicology, clinical epidemiology and health professional education. 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 Lifeinthefastlane.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.

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