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Updated: Dec 15 2021

Secondary Polycythemias

  • Introduction
    • Polycythemia results from increased hemoglobin or hematocrit
      • definition
        • hemoglobin > 16.5 g/dL in men or > 16.0 g/dL in women
        • hematocrit > 49% in men or > 48% in women
      • types of polycythemia
        • relative polycythemia
          • normal red blood cell (RBC) mass with decreased plama volume
        • absolute polycythemia
          • increased RBC mass with normal plasma volume
            • primary
              • due to mutation in RBC progenitor cells resulting in proliferation
            • secondary
              • due to elevated erythropoietin (EPO)
    • This topic focuses on secondary polycythemia
      • Secondary Polycythemia
      • Etiology
      • Pathology
      • Hypoxia
      • Cardiopulmonary disease:
      • cyanotic heart disease
      • chronic pulmonary disease
      • obstructive sleep apnea
      •  High altitude
      •  Carbon monoxide toxicity
      • Smoking
      • Combination of hypoxia, lung disease, carbon monoxide toxicity, and volume contraction
      • Renal disease
      • Kidneys sense decreased oxygen levels:
      • reduced blood flow to the kidneys
      • (renal artery stenosis)
      • intrinsic renal disorder
      • (renal cysts)
      • (hydronephrosis)
      •  Renal transplantation
      • Tumor
      • Due to autonomous production of EPO
      •  Hepatocellular carcinoma
      •  Renal cell carcinoma
      •  Pheochromocytoma
      •  Hemangioblastoma
      •  Uterine leiomyomata
      • Other
      • Blood "doping"
      •  Self-injection of EPO
      •  Anabolic steroid use
  • Studies
    • Peripheral blood smear
      • rule out other pathologies
    • Serum EPO
      • elevated
        • suggests secondary polycythemia
      • absent or low
        • suggests primary polycythemia
    • Complete blood count
      • elevated hemoglobin and/or hematocrit
    • Urinalysis and creatinine
    • Electrolytes
    • Liver function test
    • Pulse oximetry
  • Differential
    • Primary polycythemia
      • key distinguishing factors
        • low or absent EPO
        • consider JAK2 mutation testing
  • Treatment
    • Medical
      • treat underlying condition
  • Complications
    • Thrombosis
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