HELLP

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Charles Gomersall

First posted July 2006

Haemolysis, Elevated Liver enzymes, Low Platelets

• Unclear whether this is a unique entity or a variant of pre-eclampsia
• Occurs in ~1/1000 pregnancies with >70% presenting antenatally

Pathogenesis

• activation of complement and coagulation cascades
• increased vascular tone
• alteration of thromboxane:prostacyclin ratio
  ⇒ endothelial injury
  ⇒ microangiopathic anaemia and platelet aggregation
  ⇒ periportal hepatic necrosis and thrombocytopaenia

Clinical features

• ± nausea & vomiting
• ± right upper quadrant pain
• ± weight gain or oedema
• ± hypertension, proteinuria and other features of pre-eclampsia/eclampsia

Diagnostic criteria

Controversial

• haemolysis (with blood film abnormalities)
  • some accept LDH>600 IU/l with hyperbilirubinaemia
  • others use LDH>164 IU/l with falling haemoglobin
• thrombocytopaenia (<100,000/ml)
  • some use <150,000 as cut-off
• elevated transaminases (AST and ALT >70 IU/l)
  • some use AST>48 and ALT>24

Complications

• disseminated intravascular coagulation
• placental abruption
• acute renal failure
• severe ascites
• pulmonary oedema & pleural effusions
• cerebral oedema
• laryngeal oedema
• hepatic infarction
• subcapsular haematoma ±rupture
• ARDS
• laryngeal oedema
• retinal detachment
• maternal death

Differential diagnosis

• acute fatty liver of pregnancy
• cholestasis of pregnancy
• appendicitis, cholecystitis, pyelonephritis, urinary stones
• gastroenteritis
• thrombotic thrombocytopaenic purpura
• idiopathic thrombocytopaenic purpura
• systemic lupus erythematosus
• haemolytic uraemic syndrome
• viral hepatitis

Management

• IV magnesium sulphate as prophylaxis against fits
• ± antihypertensives
  • methyldopa for mild hypertension
  • hydrallazine or labetalol for more severe cases

Corticosteroids

• limited prospective data suggests that dexamethasone increases platelet counts and urine output and decreases LDH and transaminases
• effect may be transient with worsening of laboratory parameters 48-72 hours after the start of treatment
• steroids also improve fetal lung maturity in those <35 weeks gestation

Delivery of fetus

The mainstay of treatment is delivery of the fetus. The optimal timing is dependent on the condition of both mother and fetus.

Antepartum management

• some advocate higher dose of dexamethasone (20 mg 6 hourly for up to 4 doses) for patients with:
  • platelet count <20,000/ml
  • CNS dysfunction (blindness or paralysis)

Postpartum management

HELLP may present postpartum or may worsen due to a rebound effect. Management consists of IV MgSO₄ and blood pressure control. Corticosteroids and transfusion for inappropriate bleeding or thrombocytopaenia with platelet count <50,000/ml.

Subcapsular haematoma

Clinical features

• usual presentation is in 2nd or 3rd trimester
• may present in immediate post partum period
• usually associated with HELLP or severe pre-eclampsia
• RUQ pain typical but may be absent
• ±referred pain to chest or shoulder or more generalized abdominal pain
• ±peritonism
• ±hepatomegaly

Investigations

• ultrasound
• CT

Management

• conservative in the absence of rupture
  • avoid trauma to liver including abdominal palpation, convulsions, vomiting
  • serial imaging of haematoma
  • consider arterial embolization for large unruptured haematoma
• surgical management for rupture, which presents with:
  • shoulder pain
  • shock
  • abdominal distension
  • respiratory difficulty
  • pleural effusion
  • fetal death

  Can be confirmed by paracentesis

Further reading

Guntupalli SR, Steingrub J. Hepatic disease and pregnancy: an overview of diagnosis and management. Crit Care Med, 2005; 33 (10, suppl): S332-9

O'Brien JM, Barton JR. Controversies with the diagnosis and management of HELLP syndrome. Clinical Obstetrics and Gynecology, 2005; 48(2):460-77

© Charles Gomersall July 2006

©Charles Gomersall, October, 2009 unless otherwise stated. The author, editor and The Chinese University of Hong Kong take no responsibility for any adverse event resulting from the use of this webpage.
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