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