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Updated: Dec 25 2021

Neonatal Polycythemia Rubra Vera

Images shot 2015-10-29 at 11.33.12 am.jpg
  • Snapshot
    • A 2-day-old newborn presents with cyanosis and irritability. His mother had gestational diabetes, which resulted in a difficult delivery due to the baby’s large size. Delivery was further complicated by delayed clamping of the umbilical cord. On physical exam, the patient has plethora and ruddiness of his face. Heelstick hematocrit was found to be 71%. Venous sampling of hematocrit was found to be 68%.
  • Introduction
    • Venoushematocrit exceeding normal values for gestational and postnatal age
      • typically hematocrit > 65%
    • Most babies are asymptomatic
    • Distinct from adult polycythemia vera, a myeloproliferative neoplastic disorder
    • Risk factors
      • delayed clamping of umbilical cord (most common)
        • ↑ transfer of placental blood to infant
      • twin-to-twin transfusion
      • placental insufficiency
      • maternal diabetes
      • maternal hypoxemia
      • macrosomia
      • maternal hypertension
    • Hyperviscosity thought to play a role in symptoms, if any
  • Presentation
    • Majority are asymptomatic
    • Symptoms/physical exam
      • GI upset
        • poor feeding
        • vomiting
      • hypoglycemia
      • cyanosis/apnea
      • plethora
      • respiratory distress
      • lethargy
  • studies
    • Venous hematocrit > 65%
    • Glucose for hypoglycemia
    • Bilirubin level
      • ↑ RBC can lead to hyperbilirubinemia
    • Observe closely for neurologic and cardiovascularsymptoms
      • may have underlying pathologic causes of polycythemia
  • Differential
    • Other causes of polycythemia vera
      • hypoxemia
  • Treatment
    • Asymptomatic
      • supportive care
      • monitor bilirubin and glucose
    • Symptomatic
      • IV hydration with glucose
        • many have hypoglycemia
      • partial exchange transfusion
        • reduces Hct without hypovolemia
        • may not improve outcomes
        • used only if symptoms worsen
  • Complications
    • Hypoglycemia
  • Prognosis
    • Unclear effect on long-term outcome
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