POLYCYTHEMIA
Presented by:
Ijaz Khan
Senior Lecturer,
NIU
Objectives :
Define polycythemia.
Types of polycythemia.
Causes or Risk factors of
polycythemia.
Symptoms of polycythemia.
Assessment and Diagnostic finding.
Treatment of polycythemia.
Define :
• Polycythemia is an increase of
red blood cells in the body. The
extra cells cause the blood to
be thicker and this in turn,
increases the risk of other
health issues, such as blood
clots.
• The term is used when
hematocrit is elevated (more
than 55% in males, and 50% in
females ). Dehydration can
cause an elevated hematocrit
but not typically to the level to
Types :
There are two types of polycythemia :
1. Primary polycythemia
2. Secondary polycythemia
Primary polycythemia is also called
polycythemia Vera(PV).
PV is a proliferative disorder of stem cells. The bone
marrow is hypercellular, and the erythrocyte,
leukocytes, and platelet counts in the peripheral blood
are often elevated.
Causes of Primary
Polycythemia
• Exact cause is unknown; considered a
clonal stem cell disorder
• Caused by mutation in hematopoietic stem
cells
• Common mutation: JAK2 gene, leading to
uncontrolled cell growth independent of
erythropoietin
• Erythropoietin levels are usually low or
normal due to negative feedback
• Mostly affects adults aged 50–70 years
• Slightly more common in men
Secondary polycythemia :
Secondary polycythemia is caused by
excessive production of erythropoietin.
The overproduction of blood cells in secondary
polycythemia is limited to the red blood cells.
Causes of secondary
polycythemia:
• Being at very high altitude.
• Obstructive sleep apnea.
• Heart or lung disease that causes a low level
of oxygen in body.
• hemoglobinopathies
Risk factors:
Some people may be more at risk than
others of primary polycythemia. The
National Center for Advancing Translation
al
Sciences(NCATS) note that most cases of
PV appear as a person ages, usually
around the age of 60 years. They also
note that it is more common in men than
in women.
PV is not necessarily hereditary, and most
people with the disease do not have a
family history of PV. However, there
appears to be a connection to a certain
Symptoms:
Symptoms results from increased blood volume
include:
• Dizziness
• Headaches
• Tinnitus
• Paresthesias
• Blurred vision
• Fatigue
Symptoms also results from increased blood
viscosity and may include :
• Angina
• Claudication
• Dyspnea
• Thrombophlebitis
Erythromelalgia : a burning sensation in
the fingers and toes, and it partially
relieved by cooling
Complications:
Possible complications of polycythemia vera
include:
Patients with polycythemia vera are at
increased risk for Thrombosis that may be
either venous or arterial. It can my reslut in
stroke or myocardial infarction. Thrombotic
complication are most common cause of
Blood clots:
Increased blood thickness and decreased blood flow, as
well as abnormalities in your platelets, raise your risk of
blood clots. Blood clots can cause a stroke, a heart
attack, or a blockage in an artery in your lungs or a vein
deep within a leg muscle or in the abdomen.
Enlarged spleen:
Your spleen helps your body fight infection and filter
unwanted material, such as old or damaged blood cells.
The increased number of blood cells caused by
polycythemia vera makes your spleen work harder than
normal, which causes it to enlarge.
Bleeding:
It is also a complication, possibly because the platelets
are often very large and somewhat dysfunctional. The
bleeding can occur in the form of nosebleeds, ulcer,
franks GI bleeding, hematuria, and intracranial
hemorrhage.
Diagnosis:
If doctors suspect that a person has polycythemia, they
will order several tests to help identify the underlying
issue.
Blood tests:
Blood tests, such as a complete blood count, will reveal
any increase in red blood cells in the bloodstream, as well
as any abnormal levels of platelets and white blood cells.
If PV seems possible, a doctor will order more specific
blood tests.
Genetic tests:
Although genetic causes of PV are more rare,
doctors may also want to analyze the person’s
bone marrow for genetic mutations that have
links to PV.
Bone marrow biopsy:
If necessary, the doctor may also want to remove
some bone marrow on which to perform tests in a
lab.
A bone marrow biopsy involves taking a small
sample of the bone marrow with a needle for
examination under a microscope.
Treatment:
Treatment for polycythemia will depend on the
underlying cause of the condition.
In people with secondary polycythemia
resulting from causes such as obstructive sleep
apnea, treating the underlying cause should
help resolve the high red blood cell count.
PV, however, is a chronic condition that has no
known cure. The treatment for PV aims to
manage the condition by reducing both the red
blood cell count and the risk of complications,
such as blood clots.
• Treatment options for PV can include:
Medical management:
Phlebotomy:
It is important part of therapy. It involves removing
enough blood(initially 500ml once or twice weekly) to
reduce blood viscosity and to deplete the patients
iron stores. Many patients are well managed by
routine phlebotomy on an intermittent basis, with the
target of maintaning the hematocrit less then 45%
• If phlebotomy alone doesn't help enough, your
doctor may suggest medications that can
reduce the number of red blood cells in your
bloodstream.
Examples include:
• Hydroxyruea
• Interferon alfa-2b
• Busulfan
• Taking low dose aspirin helps reduce the risk of
clotting in many people with PV, and it may also
alleviate troubling symptoms, such as headaches.
• Doctors may recommend antihistamine drugs to
help relieve itchiness.
Nursing management:
• Monitor for cerebral and peripheral thrombosis.
• Assist the patient for ambulation.
• Perform phlebotomy as per doctors orders.
• Administer iv fluids and encourage to take oral
fluids.
• Administer pain management measures.
• Advice to do regular exercise.
Nursing Diagnosis
• Ineffective tissue perfusion related to increased
blood viscosity as evidenced by dizziness, weak
peripheral pulses, and numbness in extremities.
• Fatigue related to increased cellular metabolism as
evidenced by patient reports of exhaustion,
exertional intolerance, and headache.
• Risk for thromboembolism related to hyperviscosity
of blood and elevated platelet count.
