- OBSTETRICAL EMERGENCY
- Rare syndrome (occurring in 0.5-0.9% of all pregnancies and in 10–20% severe preeclampsia)
Spiral arteries fail to remodel because of inadequate trophoblast invasion or defective endothelial apoptosis result in ischemia of the placenta. This causes activation of endothelium, which is accompanied by an increased release of antiangiogenic factors to cause hypertension and proteinuria. It may lead to multiorgan microvascular injury, which is the reason for liver damage in HELLP syndrome.
Microangiopathic haemolytic anaemia (MAHA):Mechanical hemolytic anemia characterized by red blood cell fragmentation or schistocytes on peripheral blood (PB) smear
Observed in various conditions such as:
- Thrombotic thrombocytopenic purpura (TTP)
- Hemolytic uremic syndrome (HUS)
- Disseminated intravascular coagulation (DIC)
- HELLP syndrome
- Systemic infection
- Immune disorders
Majority (~70%) present in the third trimester (27-37 weeks) while the remainder within 48 hours after delivery.
Symptoms usually continuously progress and intensity often changes spontaneously, characterized by exacerbation during the night and recovery during the day.
Classical features:Upper abdominal pain may be fluctuating, colic-like
- Abdominal pain (right upper abdominal quadrant or epigastric pain)
- Nausea and vomiting
- History of malaise (some days before presentation)
- Headache (30–60%)
- Visual symptoms (20%)
- Abruptio placenta, DIC (M/C cause of maternal death in HELLP)
- Acute renal failure (84%)
- Severe ascites
- Pulmonary oedema (6%, 44% if renal failure present)
Perinatal complications:↑ Mortality & morbidity
- Preterm delivery
- Tennessee classification system (diagnosis)
- Mississippi-Triple class system (grading)
Lab investigations:Triad signs of haemolysis, elevated liver enzymes and thrombocytopenia
- Thrombocytopenia (platelet < 1 lakh/mm3)
- ALT & AST > 70 IU/l
- LDH > 600 IU/l
- Bilirubin > 1.2 mg/dl
Peripheral blood smear (PBS):Hemolysis due to microangiopathic haemolytic anaemia (MAHA)
- Schizocytes: Fragmented RBCs
- Burr cells: Contracted RBCs with spicula (Burr cells)
- Polychromatic RBCs
CT scan (abdomen):
- Subcapsular liver hematoma
- Pre-eclampsia: Normal liver enzymes and platelet count. Schistocytes also absent.
- Acute fatty liver of pregnancy (AFLP): Hypoglycemia present in AFL but absent in HELLP
- Thrombotic thrombocytopenic purpura (TTP): Usually manifests in 2nd/3rd trimester, and liver abnormalities are not as elevated as in HELLP syndrome. Patients typically normotensive and have undetectable ADAMTS 13 activity.
- Hemolytic-uremic syndrome (HUS): Same findings as TTP and patients have signs of renal failure
- Lupus flare: Liver pathology absent in lupus.
- Antiphospholipid syndrome (APS): Dominant features are arterial/venous thrombosis and repeated pregnancy loss
- Other: Viral hepatitis, cholecystitis, cholangitis, gastritis, gastric ulcer, acute pancreatitis, upper UTI.
Immediate termination of pregnancy:Irrespective of gestational age; DEFINITIVE MANAGEMENT
- Antiseizure prophylaxis: MgSO4
- Thrombocytopenia: Platelet, FFP, plasmapheresis
Corticosteroids(not useful in HELLP)