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Female Reproductive System ORGAN SYSTEMS

HELLP syndrome

Serious complication in pregnancy characterized by (H) haemolysis, (EL) elevated liver enzymes and (LP) low platelet count.

Preeclampsia is classically defined as the new onset of hypertension and proteinuria after 20 weeks of gestation. | Preeclampsia: Pathophysiology: Jorge Muniz, PA-C

Serious complication in pregnancy characterized by (H) haemolysis, (EL) elevated liver enzymes and (LP) low platelet count.

  • OBSTETRICAL EMERGENCY
  • Rare syndrome (occurring in 0.5-0.9% of all pregnancies and in 10–20% severe preeclampsia)
Schematic representation of the events leading to the different hypertensive disorders of pregnancy
Schematic representation of the events leading to the different hypertensive disorders of pregnancy. | DM: diabetes mellitus. SLE: systemic lupus erythematodes. APLS: anti-phospholipid syndrome.| W. Ganzevoort, A. Rep, G.J. Bonsel, J.I. de Vries, H. Wolf Dynamics and incidence patterns of maternal complications in early-onset hypertension of pregnancy BJOG., 114 (2007), pp. 741-750

Pathophysiology

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

Presentation

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

Other features:

  • History of malaise (some days before presentation)
  • Headache (30–60%)
  • Visual symptoms (20%)

Complications

Maternal complications

  • 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)
  • ARDS
  • Sepsis
  • Death

Perinatal complications:

↑ Mortality & morbidity
  • Preterm delivery
  • RDS
  • Sepsis

Diagnosis

Diagnostic criteria:

  • Tennessee classification system (diagnosis)
  • Mississippi-Triple class system (grading)
Main diagnostic criteria of the HELLP syndrome
Main diagnostic criteria of the HELLP syndrome | Haram, K., Svendsen, E., & Abildgaard, U. (2008). Author ‘ s response to reviews Title : The HELLP syndrome : clinical issues and management.

Lab investigations:

Triad signs of haemolysis, elevated liver enzymes and thrombocytopenia
  • Thrombocytopenia (platelet < 1 lakh/mm3)
  • LFT
    • 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
Peripheral blood smear: HELLP syndrome
Schistocytes, polychromasia and nucleated RBC (arrow) in peripheral blood (Wright stain, ×1,000). | Park J. (2018). Microangiopathic hemolytic anemia as initial presentation of recurrent colon cancer. Blood research, 53(2), 167–169. https://doi.org/10.5045/br.2018.53.2.167

CT scan (abdomen):

  • Subcapsular liver hematoma
CT in HELLP syndrome showing subcapsular liver hematoma
Abdominal computed tomography showing subcapsular liver hematoma | Lam, M., & Dierking, E. (2017). Intensive Care Unit issues in eclampsia and HELLP syndrome. International journal of critical illness and injury science, 7(3), 136–141. https://doi.org/10.4103/IJCIIS.IJCIIS_33_17

Differential diagnosis:

  • 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.

Management

Immediate termination of pregnancy:

Irrespective of gestational age; DEFINITIVE MANAGEMENT

Supportive management:

  • Antiseizure prophylaxis: MgSO4
  • Thrombocytopenia: Platelet, FFP, plasmapheresis
  • Corticosteroids (not useful in HELLP)

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