3. DEFINITION :
It is the production & presence of increased number of RBC’s in
blood.
It is an abnormally increased concentration of Hemoglobin in blood
either through reduction of plasma volume or increased blood
viscosity.
It is a laboratory finding in which Hematocrit or Hemoglobin
concentration are increased in the blood.
It is a blood disorder occurring when there are too many red blood
cells in the circulation.
4.
5. INCIDENCE :
The median age of onset is 65 years, but can occur at any age.
Survival rate is more in patients who diagnosed before the age of 60
years.
It is estimated to affect 2.8 per 1,00,000 population in men & 1.3 per
1,00,000 population in women.
6. ETIOLOGY & RISK FACTORS :
Idiopathic
Mutation
Obstructive Sleep Apnea
COPD
Hypoventilation syndrome
Long term Smoking
Genetic Predisposition
Living in High Altitudes
Long term exposure to carbon Monoxide (Tunnel workers, Car
Garage workers)
7. CLASSIFICATION :
1. Primary Polycythemia or Polycythemia Vera
2. Secondary Polycythemia
3. Relative Polycythemia
4. Stress Polycythemia
8. POLYCYTHEMIA VERA:
It is a myeloproliferative disorder arising from chromosomal
mutation in single pluripotent stem cell.
Hence, the cells involved are not only RBC’s, but also granulocytes &
platelets.
It leads to increased production of each of blood cells, which is
exhibited in peripheral blood sample.
Since the bone marrow is hypercellular, excessive red blood cells are
found.
9. SECONDARY POLYCYTHEMIA :
Chronic hypoxia causes secondary polycythemia.
Hypoxia stimulates Erythropoietin production in the kidney, which in
turn promotes erythrocyte production.
Causes may include Cardiovascular disease, Alveolar
Hypoventilation, defective Oxygen transport or tissue hypoxia.
It is a physiologic response in which the body tries compensate for a
problem.
10. RELATIVE POLYCYTHEMIA:
Here number of RBC will increase without any change in the total
cellular mass.
It is caused due to loss of plasma volume with resultant hemo –
concentration, as seen in severe dehydration related to vomiting &
diarrhoea.
11. STRESS POLYCYTHEMIA :
It is a term applied to chronic state of low plasma volume which is
seen commonly in active, hard working, anxious, middle aged man.
Here, red blood cell volume is normal but plasma volume is low.
12. CLINICAL MANIFESTATIONS :
Circulatory features occur due to hypervolemia and hyperviscosity.
It includes subjective symptoms like Headache, Vertigo, Parasthesia,
Dizziness, Tinnitus and Visual disturbances.
Patients may also express Angina, hypoxia, intermittent claudication
and thrombophlebitis.
Hemorrhagic Features caused due to vessel rupture from
overdistension or inadequate platelet function.
• It include Petechiae, Ecchymoses, Epistaxis, GI Bleeding.
13. Other features :
Hepatomegaly & Splenomegaly
Hyperuricemia
Gout
Erythromelalgia - a burning sensation in fingers and toes.
Generalized Pruritus
Fatigue
Sleep disturbances
14. Diagnostic Evaluation :
History collection & Physical Examination
Blood Examination reveals :
- Elevated Hemoglobin level
- Increased WBC Count with basophilia
- Elevated platelets with dysfunction
- Elevated leukocyte alkaline phosphatase
- Increased histamine level
Bone Marrow Examination
Chest X – Ray
Electrocardiogram
Echocardiogram
15. MANAGEMENT :
Treatment directed towards reducing blood volume, blood viscosity &
bone marrow activity.
1. Phlebotomy
• It is the first option of treatment in this condition.
• It involves removing enough blood from body (initially 500 ml once
or twice in a week) to diminish blood volume until the desired
hematocrit value is achieved.
• Patient managed with repeated phlebotomies becomes iron deficient &
experience depletion in iron store.
• Iron supplementation should be avoided.
16. 2.Hydration therapy is used to reduce blood’s viscosity.
3. Myelosuppressive agents
Medications like Busulfan, Hydroxyurea (hydrea), Melphalan (Alkeran)
are used to inhibit bone marrow activity.
4. Antiplatelet agents like Aspirin, Anagrelide, Dipyridamole are used
to prevent thrombotic complications.
5. Interferon Alpha is used to stimulate immune system to fight over
production of Red Blood cells.
6. Antihistamines or Ultraviolet light therapy is used to relieve
discomfort in case of Itching.
17. LIFE STYLE MODIFICATIONS :
1. Exercise
Regular moderate exercises such as brisk walking, stretching of legs &
ankles, Range of motion exercises can improve blood flow and reduce
the risk of clots in circulation.
2. Avoid Tobacco Use
Smoking or any other form of tobacco use can cause peripheral
vasoconstriction and increase the risk of cardiac problems.
Continued…
18. 3. Be Good to your Skin
• To reduce itching, bath in cool water, pat your skin dry, avoid hot tubs,
heated whirlpools and hot showers.
• Try not to scratch as it may damage your skin.
4. Avoid Extreme Temperature
• Poor blood flow can increase the risk of many complications.
• In cold weather, always wear warm clothing, particularly on hands &
feet.
• In hot weather, protect yourself from sun & drink plenty of water.
19. NURSING MANAGEMENT :
1. Impaired tissue perfusion related to phlebotomy procedure as
manifested by cyanosis.
2. Impaired breathing pattern related to decreased level of RBC in blood
as evidenced by dyspnea.
3. Acute pain related to venipuncture as evidenced by verbalization.
4. Imbalanced nutrition, less than body requirement related to restriction
on diet as manifested by weight loss.
5. Anxiety related to disease condition & treatment as evidenced by
frequent doubts.