Polycythemia is a stem cell disorder characterized by an increased number of red blood cells and hemoglobin concentration in the blood. There are three types: primary polycythemia (polycythemia vera), secondary polycythemia, and relative polycythemia. Polycythemia vera involves uncontrolled growth of myeloid stem cells leading to elevated red blood cell, white blood cell, and platelet counts. Treatment goals are to reduce blood viscosity and bone marrow activity through phlebotomy, myelosuppressive drugs, radiation therapy, or interferon alpha 2b.

2. POLYCYTHEMIA

3.  Polycythemia is defined as an increase in both the
number of circulating erythrocytes and the
concentration of hemoglobin within the blood
 It is a stem cell disorder characterized by pan
hyperplastic, malignant and neoplastic marrow
disorder.

4. CLASSIFICATION
 Primary polycythemia or polycythemia vera
 Secondary polycythemia
 Relative polycythemia

5. POLYCYTHEMIA VERA
 Polycythemia Vera or primary polycythemia is a
proliferative disorder in which myeloid stem cells seem to
have escaped normal control mechanisms.
 Then bone marrow is hypercellular and the erythrocytes
leukocytes and platelet counts in the peripheral blood are
elevated however the erythrocytes elevation is
predominant and the
 Hematocrit can exceed 60%

6. ETIOLOGY
 Primary acquired or inherited genetic mutations
 secondary underlying medical problems
 chronic hypoxia
 peak incidence in the age group 50 to 70 years of age
 underlying causes unknown
 survival rate is ten to 20 years living in high altitudes

10.  IMAGING STUDIES:
 USG
 CT
 Which shows posteriorly enlarged spleen in obese
people

11. MEDICAL MANAGEMENT
 Permanent cure is un Available
 but remission for many years can be achieved
 the goals of care in polycythemia Vera are
 reduction of blood volume viscosity
 bone marrow activity
 these are accomplished through
 phlebotomy
 administration of myelosuppressive agents
 radiation therapy
 interferon alpha 2B

12. phlebotomy
 It is used to normalize red cell mass as quickly as possible it
involves removing enough blood to diminish the blood viscosity.
(initially 500 mill ones or twice a week)
 once normal hematocrit levels are reached subsequent phlebotomies
should be carried out frequently mostly monthly as necessary to
maintain the hematocrit level at about 45%
 iron deficiency will likely result due to this procedure

13. MYELOSUPPRESSIVE AGENTS
 The myelosuppressive agent hydroxyurea is commonly
used in client over 50 years of age
 Radioactive phosphorus chlorambucil busulfan and
melfalan are also tried but not indicated for long term use
because of the increased incidence of acute leukemia after
15 years

14. RADIATION THERAPY
 Radiation therapy to reduce the production of red
blood cells in the marrow

15. Interferon Alpha
 Interferon Alpha 2b is the most effective treatment for
managing pruritus associated with polycythemia Vera