Polycythemia
Represented by
Attiya Bibi
Ghose Ahmad
Ayesha Arshad
Introduction to Polycythemia
• The word "polycythemia" is derived from Greek words :
• 1. "Poly-" meaning many or much
• 2. "-cythemia" from "kytos" meaning cell and "-emia"
referring to blood
• So, polycythemia refers to a condition of having too many
red blood cells in the blood.
Polycythemia
• Polycythemia refers to an elevated level of erythrocytes, as
indicated by an increased hematocrit (more than 55% in males
and more than 50% in females).
• Total red blood cell (RBC) count in polycythemia will often
exceed 6 million cells/µl in males and 5.5 million cells/µl in
females.
• A hematocrit higher than these thresholds is considered
polycythemia.
• Polycythemia can either be primary (due to bone marrow
issues) or secondary (due to other conditions such as low
oxygen levels).
Normal range:
• Normal hematocrit range:
• Males: 40-54%
• Females: 36-48%
• Polycythemia ranges:
• Males: >55%
• Females: >50%
Causes of Polycythemia:
• There are two causes of polycythemia
• Primary: Polycythemia Vera, Myeloproliferative disorders
• Secondary: Chronic hypoxia (e.g.. COPD, heart disease),
tumors (renal cell carcinoma), living at high altitudes, and
hemoglobinopathies.
Primary Polycythemia:
• Primary Polycythemia is also known as Polycythemia Vera (PV).
• PV is a myeloproliferative disorder characterized by the excessive
production of red blood cells, predominantly affecting erythrocytes. It
results in Increased blood viscosity, which may lead to thrombotic
complications
• Triggers:
• 1. JAK2 mutation
• 2. Hypersensitivity to erythropoietin
• 3. Chronic inflammation
• 4. High platelet counts
• 5. Splenic enlargement
Pathophysiology of Polycythemia
Vera
Polycythemia vera is a myeloproliferative disorder where there is
uncontrolled proliferation of erythroid precursors In the bone marrow.
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This leads to an increase in red blood cell mass, which thickens the blood
and increases viscosity.
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The mutation in the JAK2 gene causes hypersensitivity to erythropoietin
(EPO), leading to excessive RBC production regardless of EPO levels.
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The increased blood volume and viscosity result in Impaired blood flow,
raising the risk of thrombosis, and may lead to complications such as
stroke, heart attack, or organ damage.
Complications:
• Thrombosis (stroke, myocardial infarction)
• Hemorrhage (GI bleeding, nosebleeds)
• Gout Myelofibrosis
• Progression to AML
Medical Management:
1. Phlebotomy to reduce blood viscosity
2. Hydroxyurea for marrow suppression
3. Anagrelide for controlling platelet count
4. Interferon alfa-2b for pruritus relief
5. Aspirin for preventing thrombotic events
Nursing Management:
1. Educate about thrombotic risks and prevention
2. Monitor for signs of bleeding
3. Advise on lifestyle changes (e.g., reducing alcohol)
4. Provide relief for pruritus
5. Educate on avoiding iron supplements
Secondary Polycythemia
• Secondary polycythemia results from elevated erythropoietin levels due
to chronic hypoxia or other causes stimulating erythropoiesis.
• This form is often reversible when the underlying cause is addressed.
• Triggers:
1. Chronic obstructive pulmonary disease (COPD)
2. Cigarette smoking
3. Cyanotic heart disease
4. High altitudes
5. Renal cell carcinoma
Pathophysiology of Secondary
Polycythemia
• Secondary polycythemia is caused by an increase in
erythropoietin (EPO) production in response to chronic hypoxia
or other stimull.
• Conditions such as chronic lung disease, living at high altitudes,
or tumors that secrete EPO lead to elevated EPO levels.
• The increased EPO stimulates the bone marrow to produce
more red blood cells to compensate for the oxygen deficiency.
• This results in an elevated RBC mass, but uniike polycythemia
vera, the increase is due to a physiological response to a known
condition rather than a genetic mutation.
Complications:
• Increased blood viscosity
• Thrombosis
• Organ damage (e.g., spleen or liver enlargement)
• Headaches
• Visual disturbances
Medical Management:
1. Treat the underlying cause (eg, oxygen therapy for COPD)
2. Phlebotomy for reducing blood viscosity
3. Erythropoietin inhibitors in rare cases
4. Smoking cessation
5. Oxygen therapy for chronic hypoxia
Nursing Management:
1. Educate on managing the underlying condition
2. Monitor hematocrit levels
3. Encourage hydration to manage viscosity
4. Provide support for smoking cessation
5. Assess for complications related to blood viscosity
