- A myeloproliferative disease that results in increased red blood cells, in addition to mild increases in leukocytes and platelets
- 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
- Low erythropoietin in primary(MCQ)
- High erythropoietin in secondary
- 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.
- 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.
- 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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