Distinguish HELLP from AFLP (Swansea), deliver, support coagulation and glucose, and watch for hepatic haematoma and acute liver failure.
At the bedside
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Stabilise and treat as severe pre-eclampsia
BP control and magnesium sulfate for HELLP.
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Work-up
FBC with smear, LDH, bilirubin, AST/ALT, glucose, coagulation including fibrinogen, ammonia, creatinine, uric acid; ultrasound liver (haematoma).
- 3
Deliver
Both HELLP (≥ 34 weeks or deteriorating) and AFLP require prompt delivery after maternal stabilisation.
- 4
Correct coagulation
Platelets > 50,000 before caesarean (transfuse if lower); FFP/cryoprecipitate for AFLP coagulopathy guided by fibrinogen and bleeding.
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Glucose
AFLP: hourly glucose; dextrose infusion (10-20%) for hypoglycaemia.
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Liver failure care
Encephalopathy, rising INR, lactate: manage as acute liver failure, contact transplant centre; plasma exchange in selected cases.
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Watch for haematoma
Right upper quadrant/shoulder pain with shock: subcapsular liver haematoma/rupture - CT, interventional radiology or surgery.
HELLP vs AFLP vs TTP/HUS
| Feature | HELLP | AFLP |
|---|---|---|
| Timing | Third trimester or postpartum | Third trimester (30-38 weeks) |
| Hypertension | Usually | ~50% |
| Hypoglycaemia | Uncommon | Common |
| Coagulopathy (raised INR, low fibrinogen) | Late/with DIC | Early and marked |
| Encephalopathy | Rare | Common |
| Platelets | Low (defining) | Normal or mildly low |
| Ammonia | Normal | Raised |
Swansea criteria for AFLP (≥ 6 without other cause)
| Clinical/lab | Lab/imaging |
|---|---|
| Vomiting | Bilirubin > 0.8 mg/dL (14 µmol/L) |
| Abdominal pain | Hypoglycaemia < 72 mg/dL (4 mmol/L) |
| Polydipsia/polyuria | Uric acid > 5.7 mg/dL (340 µmol/L) |
| Encephalopathy | WBC > 11,000/mm³ |
| Ascites or bright liver on ultrasound | AST/ALT > 42 U/L |
| Microvesicular steatosis on biopsy | Ammonia > 47 µmol/L; creatinine > 1.7 mg/dL (150 µmol/L); PT > 14 s or aPTT > 34 s |
14 criteria in total.
References
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Pathophysiology
HELLP is a severe form of pre-eclampsia with microangiopathic haemolysis and periportal hepatic necrosis. AFLP is linked to fetal fatty-acid oxidation defects (LCHAD deficiency), with toxic fetal metabolites causing maternal microvesicular steatosis and mitochondrial dysfunction.
Evidence
- ACOG 2020 defines HELLP and management within pre-eclampsia [ACOG 2020].
- Swansea criteria (prospective Welsh study) for AFLP diagnosis [Ch'ng 2002].
Controversies
- Corticosteroids to raise platelets in HELLP (not shown to improve maternal outcomes); plasma exchange in AFLP.
🇮🇳 India notes
- Acute viral hepatitis E is a major cause of liver failure in pregnancy in India and must be distinguished from AFLP and HELLP.
- Test newborns of AFLP mothers for LCHAD deficiency where available.
References
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