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.
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
7.
8.
9.
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