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Differential Diagnosis of Polycythemia Vera

Differential Diagnosis of Polycythemia Vera. True / Absolute Polycythemia. Apparent / Relative Polycythemia. Either a decrease in plasma volume (relative polycythemia ) or a misperception of what constitutes the upper limit of normal values for either hemoglobin or hematocrit.

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Differential Diagnosis of Polycythemia Vera

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  1. Differential Diagnosis of Polycythemia Vera

  2. True / Absolute Polycythemia Apparent / Relative Polycythemia Either a decrease in plasma volume (relative polycythemia) or a misperception of what constitutes the upper limit of normal values for either hemoglobin or hematocrit Either a clonalmyeloproliferativedisorder (polycythemiavera) or a nonclonal increase in red blood cell mass that is often mediated by erythropoietin (secondary polycythemia) Fauci, et al: Harrison’s Principles of Internal Medicine, 17th ed. Goldman: Cecil Medicine, 23rd ed.

  3. APPARENT POLYCYTHEMIA • Relative polycythemia • Conditions that cause acute depletion of plasma volume e.g. severe dehydration • The existence of chronic contraction of the plasma volume, such as postulated for: • Gaisböck'ssyndrome – relative polycythemia associated with hypertension and nephropathy • Stress / spurious polycythemia – relative polycythemia associated with emotional stress Fauci, et al: Harrison’s Principles of Internal Medicine, 17th ed. Goldman: Cecil Medicine, 23rd ed.

  4. ABSOLUTE / TRUE POLYCYTHEMIA    • Polycythemiavera • Secondary polycythemia • Congenital    • Associated with high or normal serum erythropoietin level    • Associated with low serum erythropoietin level • Acquired    • Erythropoietin mediated • Hypoxia-driven • Hypoxia-independent (pathologic erythropoietin production) • Drug associated • Unknown mechanism Fauci, et al: Harrison’s Principles of Internal Medicine, 17th ed. Goldman: Cecil Medicine, 23rd ed.

  5. Secondary Polycythemia • Congenital    • Associated with high or normal serum erythropoietin level    • Associated with low serum erythropoietin level • Acquired    • Erythropoietin mediated • Hypoxia-driven • Hypoxia-independent (pathologic erythropoietin production) • Drug associated • Unknown mechanism Fauci, et al: Harrison’s Principles of Internal Medicine, 17th ed. Goldman: Cecil Medicine, 23rd ed.

  6. Secondary Polycythemia • Congenital    • Associated with high or normal serum erythropoietin level    • Associated with low serum erythropoietin level • Acquired    • Erythropoietin mediated • Hypoxia-driven • Hypoxia-independent (pathologic erythropoietin production) • Drug associated • Unknown mechanism Fauci, et al: Harrison’s Principles of Internal Medicine, 17th ed. Goldman: Cecil Medicine, 23rd ed.

  7. Secondary Polycythemia: Congenital Fauci, et al: Harrison’s Principles of Internal Medicine, 17th ed. Goldman: Cecil Medicine, 23rd ed.

  8. Secondary Polycythemia • Congenital    • Associated with high or normal serum erythropoietin level    • Associated with low serum erythropoietin level • Acquired    • Erythropoietin mediated • Hypoxia-driven • Hypoxia-independent (pathologic erythropoietin production) • Drug associated • Unknown mechanism Fauci, et al: Harrison’s Principles of Internal Medicine, 17th ed. Goldman: Cecil Medicine, 23rd ed.

  9. Secondary Polycythemia: Acquired Fauci, et al: Harrison’s Principles of Internal Medicine, 17th ed. Goldman: Cecil Medicine, 23rd ed.

  10. Secondary Polycythemia • Congenital    • Associated with high or normal serum erythropoietin level    • Associated with low serum erythropoietin level • Acquired    • Erythropoietin mediated • Hypoxia-driven • Hypoxia-independent (pathologic erythropoietin production) • Drug associated • Unknown mechanism Fauci, et al: Harrison’s Principles of Internal Medicine, 17th ed. Goldman: Cecil Medicine, 23rd ed.

  11. Secondary Polycythemia: Acquired Fauci, et al: Harrison’s Principles of Internal Medicine, 17th ed. Goldman: Cecil Medicine, 23rd ed.

  12. Secondary Polycythemia • Congenital    • Acquired    • Erythropoietin mediated • Hypoxia-driven • Hypoxia-independent (pathologic erythropoietin production) • Drug associated • Erythropoietin doping • Treatment with androgen preparations    • Unknown mechanism   Fauci, et al: Harrison’s Principles of Internal Medicine, 17th ed. Goldman: Cecil Medicine, 23rd ed.

  13. Secondary Polycythemia • Congenital    • Acquired    • Erythropoietin mediated • Hypoxia-driven • Hypoxia-independent (pathologic erythropoietin production) • Drug associated • Unknown mechanism   • Post–renal transplant erythrocytosis Fauci, et al: Harrison’s Principles of Internal Medicine, 17th ed. Goldman: Cecil Medicine, 23rd ed.

  14. References • Fauci, et al: Harrison’s Principles of Internal Medicine, 17th ed. • Goldman: Cecil Medicine, 23rd ed.

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