Polycythemia vera

    • A myeloproliferative disease that results in increased red blood cells, in addition to mild increases in leukocytes and platelets
    • Etiology
      • Primary PV: A primary bone marrow cause (low erythropoietin)
      • Secondary PV (other causes that result in increased erythropoietin) (MCQ)
        • Hypoxia (high altitudes, lung disease)
        • Smoking (due to carboxyhemoglobin)
        • Renal cell carcinoma
    • Males more commonly affected (MCQ)
    • More common in people > 60
    • Associated with high risk of cerebrovascular accidents (hypercoagulation), MIs, and bleeding(MCQ)
    • Risk of progression to leukemia or myelofibrosis (MCQ)
    • Signs and symptoms
      • Pruritus, Plethora
      • Splenomegaly ,Epistaxis (MCQ)
      • Neuro symptoms such as vision changes
    • Diagnosis
      • Low erythropoietin in primary(MCQ)
      • High erythropoietin in secondary
    • Treatment
      • Serial phlebotomy to decrease blood volume
      • Consider hydroxyurea for myelosuppression (in primary) (MCQ)
      • Aspirin to prevent thrombosis(MCQ)
    • Clinical Pearls :
      • 2° polycythemia is associated with hypernephroma, cerebellar hemangioma, hepatoma, and giant uterine myomas. (MCQ)
      • Bone marrow fibrosis is a late complication of polycythemia vera.
      • Increased red blood cell mass.
      • Splenomegaly is seen in PV(MCQ)
      • Normal arterial oxygen saturation. is seen in PV(MCQ)
      • A mutation in JAK2, a signaling molecule, has been demonstrated in 95% of cases(MCQ)
      • Common complaints related to expanded blood volume and increased blood viscosity are headache, dizziness, tinnitus, blurred vision, and fatigue
      • Generalized pruritus (Aquagenic pruritis) (MCQ)
        • especially following a warm shower or bath
        • related to histamine release from the basophilia.
      • Epistaxis
        • related to engorgement of mucosal blood vessels in combination with abnormal hemostasis due to qualitative abnormalities in platelet function.
      • median age at presentation is 60 years.
      • Thrombosis
        • most common complication of polycythemia vera (MCQ)
        • major cause of morbidity and death in this disorder.
        • It is related both to increased blood viscosity and abnormal platelet function..
        • Paradoxically, in addition to thrombosis, increased bleeding also occurs.
      • There is a high incidence of peptic ulcer disease(MCQ)
      • The hallmark of polycythemia vera is a hematocrit above normal, at times greater than 60%.(MCQ)
      • Red blood cell morphology is normal
      • The bone marrow is hypercellular, with panhyperplasia of all hematopoietic elements
        • bone marrow examination is usually not necessary to establish the diagnosis.
      • Iron stores are usually absent from the bone marrow, having been transferred to the increased circulating red blood cell mass.
      • Spurious polycythemia(MCQ)
        • an elevated hematocrit is due to contracted plasma volume rather than increased red cell mass,
        • related to diuretic use or may occur without obvious cause.
      • One unit of blood (approximately 500 mL) is removed weekly until the hematocrit is less than 45%;
      • Anagrelide may be substituted or added when hydroxyurea is not well tolerated but is not the preferred initial agent. (MCQ)
      • Over time, polycythemia vera may convert to myelofibrosis or to chronic myeloid leukemia. (MCQ)

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