Hellp Syndrome

Description

• HELLP syndrome:
  • Hemolysis
  • Elevated Liver Enzymes
  • Low Platelets
• No universally accepted definition of HELLP
• Continuum with severe preeclampsia as most patients will be hypertensive
• Liver involvement is hallmark:
  • Other organs may be involved (e.g., brain, kidneys, lungs)
• Most maternal deaths occur with severe preeclampsia
• Increased mortality rate is associated with hepatic hemorrhage or CNS or vascular insult to the cardiopulmonary or renal systems
• Incidence: 0.2% of all pregnancies
• 12–18% have normal BP
• Occurs in 20% of pregnancies with severe preeclampsia or eclampsia
• At diagnosis:
  • 52% preterm
  • 18% term
  • 32% postpartum

Risk Factors

Frequently white, multiparous, older
Pediatric Considerations
Infant mortality is greater in women with HELLP

Etiology

• Unclear, but linked to the etiology of preeclampsia which has 4 proposed etiologies:
  • Abnormal trophoblastic invasion of uterine vessels
  • Immune reaction to maternal, paternal, and fetal tissues
  • Maternal response to cardiovascular or inflammatory changes of pregnancy
  • Genetic factors
• Proposed mechanism of HELLP:
  • Fetal–placental debris is released into maternal circulation, causing systemic inflammatory response
  • Vascular constriction causes resistance to blood flow and HTN
  • Vasospasm probably damages vessels directly
  • Endothelial cell is damaged and interendothelial cell leaks are the result
• Small-vessel leaks:
  • Platelets and fibrinogen get deposited in the subendothelium
  • Fibrin deposition develops in severe cases
• Vascular changes and local tissue hypoxia lead to hemorrhage, necrosis, and end-organ damage