POLYCYTHEMIA Presented By: Mr. Nandish.S Asso. Professor Mandya Institute of Nursing Sciences
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 H ematocrit or H emoglobin concentration are increased in the blood. It is a blood disorder occurring when there are too many red blood cells in the circulation.
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.
ETIOLOGY & RISK FACTORS : Idiopathic Mutation Obstructive Sleep A pnea COPD Hypoventilation syndrome Long term Smoking Genetic Predisposition Living in High Altitudes Long term exposure to carbon Monoxide (Tunnel workers, Car Garage workers)
CLASSIFICATION : Primary Polycythemia or Polycythemia Vera Secondary Polycythemia Relative Polycythemia Stress Polycythemia
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.
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.
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 h emo – concentration, as seen in severe dehydration related to vomiting & diarrhoea.
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.
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.
Other features : Hepatomegaly & Splenomegaly Hyperuricemia Gout Erythromelalgia - a burning sensation in fingers and toes. Generalized Pruritus Fatigue Sleep disturbances
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 E xamination Chest X – Ray Electrocardiogram Echocardiogram
MANAGEMENT : Treatment directed towards reducing blood volume, blood viscosity & bone marrow activity. 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.
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.
LIFE STYLE MODIFICATIONS : 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 U se Smoking or any other form of tobacco use can cause peripheral vasoconstriction and increase the risk of cardiac problems. Continued…
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 T emperature 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.
NURSING MANAGEMENT : Impaired tissue perfusion related to phlebotomy procedure as manifested by cyanosis. Impaired breathing pattern related to decreased level of RBC in blood as evidenced by dyspnea . Acute pain related to venipuncture as evidenced by verbalization. Imbalanced nutrition, less than body requirement related to restriction on diet as manifested by weight loss. Anxiety related to disease condition & treatment as evidenced by frequent doubts.