POLYCYTHEMIA

Learning objectives
By the end of the class , the students will be able to

 Explain the brief Physiology of Blood forming organs
 Define the term polycythemia
 Enumerate the etiology
 Classify the types of polycythemia
 Enlist the clinical manifestations
 Mention the diagnostic tests
 Describe the management
 Narrate the complications

Overview
 Anatomy and physiology of RBC
 Definition – polycythaemia
 Etiology
 Types of polycythaemia
 Clinical manifestations
 Diagnostic tests
 Management
 Complications
Introduction
Introduction
• Polycythemia vera is a chronic myelo proliferative
disorder characterized by increased red blood cell mass
(RCM), or erythrocytosis
• The resultant hyperviscosity of the blood predisposes
such patients to thrombosis
Review of Hematologic System
 Hematology is the study of the blood forming tissues.
 In fetal life ,various tissues produce red blood cells but

after birth their production is controlled, exclusively by
the bone marrow, primarily in the long bones of the
vertebrae, sternum and ribs assume RBC production.
 Age, sex and the altitude at which a person lives affect
the number of RBCs.
Contd…..
Bone marrow:
Hematopoiesis (red blood cell production) occurs in the

bone marrow during the first 5 years of life. Beyond 5
years of age ,most red blood cells are produced in the
marrow of the ribs, sternum, vertebrae, pelvis, skull,
scapulae and clavicle.
Constitution of Blood
Definition
Polycythemia is an increase in hematocrit,

hemoglobin or red blood cell count above the normal
limits either through reduction of plasma volume or
increase in red cell numbers. The Number of RBC is 4.2
to 6.2Millions/ cu.mm
Incidence
• Polycythemia vera is a rare disease
• The peak incidence of PV is age 50-70 years
– However, it occurs in persons of all age groups,

including those in early adulthood and childhood,
albeit rarely.
• The disease is slightly more common in males than in
females.
Etiology
• Chronic hypoxia
• Long-term cigarette smoking
• Familial and genetic predisposition

• Living in high altitudes
• Long-term exposure to carbon monoxide (tunnel workers, car
garage attendants, residents of highly polluted areas)
• Neoplasm
• Steroids
Types of Polycythaemia
• Primary polycythemia
• Secondary polycythemia
• Relative polycythemia
• Stress polycythemia
Primary Polycythemia
• Primary Polycythemia occurs when excess red blood

cells are produced as a result of an abnormality of the
bone marrow.
• Often, excess white blood cells and platelets are also
produced
Secondary polycythemia
• Secondary polycythemia is usually due to increased

erythropoietin (EPO) production either in response to
chronic hypoxia (low blood oxygen level) or from an
erythropoietin secreting tumor
Relative polycythemia
• Relative erythrocytosis is an increase in RBC numbers

without an increase in total RBC mass.
• Usually, this is caused by loss of plasma volume with
resultant hemo-concentration, as seen in severe
dehydration related to vomiting and diarrhea.
Stress polycythemia
• Stress polycythemia is a term applied to a chronic (long

standing) state of low plasma volume which is seen
commonly in active, hard working, anxious, middle-aged
men. In these people, the red blood cell volume is normal,
but the plasma volume is low
Clinical Manifestations
Symptoms results from increased blood volume:

• Cyanosis
• Reddened face with engorged retinal veins
• Itching after bath

• Feeling of fullness in head with headache
• Weakness, fatigue and dizziness
• Tinnitus (ringing or buzzing in the ears.)

• Parasthesia, numbness, burning or weakness in hands
and legs.
• Visual disturbances
• Nose bleeding
• Abdominal bloating
Symptoms from Increased Viscosity:

• Angina
• Dyspnea
• Hypoxia
• Bone and joint pain
• Thrombophlebitis
Other Symptoms
• Weight loss
• Breathing difficulty when lying down
• Chronic cough
• Night sweats and sleep disturbances
• Burning sensation over fingers or toes
• Splenomegaly and hepatomegaly
• Formation of blood clots in the blood vessels

Diagnostic Test
History Collection And Physical Examination:
Questions about
• Smoking,
• Living at high altitudes,
• Breathing difficulty,
• Sleep disturbances
• Chronic cough
Diagnostic test
Diagnostic test
Blood Tests
The blood studies will show

• An increase in the number of red blood cells
• Elevated hematocrit measurement (>50%)
• Elevated levels of hemoglobin (>18 g/dl)
• Very low levels of erythropoietin

Diagnostic Test
• PV should be suspected when hemoglobin and/or

hematocrit levels are elevated
– (> than 18 g per dL [180 g per L] in white men and >
than 16 g per dL [160 g per L] in blacks and women)
• hematocrit level greater than 52 percent (0.52) in white
men and 47 percent (0.47) in blacks and women
Diagnostic test
Diagnostic Test
BONE MARROW ASPIRATION OR BIOPSY
If an examination of the bone marrow shows that it's

producing higher than normal numbers of blood cells, it
may be a sign of polycythemia.
Diagnostic Test
• Chest X-Ray
• Electrocardiogram
• Echocardiogram
• SpO2 measurement
Criteria for Diagnosis
• Major Criteria
– Total RBC vol
• Men > or = to 36 mL/kg
• Women > or = to 32 mL/kg
– Arterial 02 saturation > or = to 92%
– Splenomegaly
Criteria for Diagnosis
• Minor Criteria
– Thrombocytosis with platelet count > 400,000/mL
– Leukocytosis with WBC > 12,000/mL
– Increased leukocyte alkaline phosphatase LAP >

100U/L (no infection)
– Serum B12 > 900 pg/mL or binding capacity UB12
BC > 2200 pg/mL
Criteria for diagnosis
• A diagnosis of polycythemia vera is made when a

patent fulfills
– all three of the major criteria or
– any two major and any two minor criteria

Management
PHLEBOTOMY
• Drawing a certain amount of blood out of the veins in a
procedure called phlebotomy is usually the first treatment
option for people with polycythemia.
• This reduces the number of blood cells and decreases your
blood volume, making it easier for your blood to function
properly.
Management
Low-dose Aspirin
The doctor may recommend that to take a low dose

of aspirin to reduce your risk of blood clots. Low-dose
aspirin may also help reduce burning pain in your feet or
hands.
Management
Medication To Decrease Blood Cells

• For people with polycythemia, who aren't helped by
phlebotomy alone, medications, such as hydroxyurea
(Droxia, Hydrea), to suppress the bone marrow's ability to
produce blood cells may be used.
• Interferon alpha may be used to stimulate the immune
system to fight the overproduction of red blood cells.
Management
The use of myelosuppressive agents such as

• Radioactive phosphorus (32P),
• Chlorambucil (leukeran),
• Busulfan (myleran),
• Pipobroman (vercyte), and
• Hydroxyurea (hydrea) in conjunction with phlebotomy has

been studied.
Management
Therapy To Reduce Itching.
If you have bother some itching, the doctor may

prescribe medication, such as antihistamines, or
recommend ultraviolet light treatment to relieve your
discomfort
Management
Splenectomy in patients with painful splenomegaly
or repeated episodes of thrombosis causing splenic
infarction
Management
Lifestyle And Home Remedies

• Exercise - Moderate exercise, such as walking, can
improve the blood flow, which decreases your risk of blood
clots.
• Leg and ankle stretches and exercises also can improve
the blood circulation.
Management
Avoid Tobacco.- Using tobacco can cause the blood

vessels to narrow, increasing the risk of heart attack or
stroke due to blood clots.
Watch for sores.- Poor circulation can make it difficult for

sores to heal, particularly on the hands and feet. Inspect
the feet regularly and tell the doctor about any sores.
Management
Avoid Extreme Temperatures.

• Poor blood flow increases the risk of injury from hot and
cold temperatures.
• In cold weather, always wear warm clothing, particularly
on your hands and feet.
• In hot weather, protect yourself from the sun and drink
plenty of liquids.
Nursing management
• Monitor for peripheral and cerebral thrombosis.
• Assist the patient for ambulation

• Perform phlebotomy as per doctors order
• Administer iv fluids and encourage to take oral fluids
• Administer pain management measures
Nursing management
• Advice to do regular exercise

• Instruct to avoid tobacco
• Advise to maintain skin hygiene

• Avoid extreme temperatures
• Provide psychological support to the patient.

Nursing diagnosis
• Impaired tissue perfusion related to blood clots,

phlebotomy as evidenced by cyanosis.
• Acute pain related to surgical intervention as evidenced
by verbalization
• Impaired breath pattern related to decreased level of RBC
in blood as evidenced by dyspnea.
Nursing diagnosis
• Imbalanced nutrition less than body requirement related to

less oral intake of food as evidenced by weight loss.
• Fear and anxiety related to outcome of disease condition
as evidenced by frequent doubts.
• Knowledge deficit regarding home care management as
evidenced by frequent doubts.
Prognosis
• The major causes of morbidity and mortality are as

follows:
– Thrombosis
– Hemorrhagic complications
– Peptic ulcer disease
– Myelofibrosis and pancytopenia
– Acute leukemia or a myelodysplastic syndrome

Complications
• Hemorrhagic complications
• Myelofibrosis and pancytopenia
• Peptic ulcer disease
• Acute leukemia or a myelodysplastic syndrome

Summary
We have discussed about the

 Anatomy and physiology of RBC
 Definition – polycythaemia
 Etiology
 Types of polycythaemia
 Clinical manifestations
 Diagnostic tests
 Management
 Complications
Conclusion
• Polycythemia is a disease state in which the hematocrit

levels elevated and an increase in the number of red blood
cells or a decrease in the volume of plasma is sometimes
called erythrocytosis.
• The emergency treatment of polycythemia (e.g., in
hyperviscosity or thrombosis) is by phlebotomy.
• Phlebotomy may also be used on a regular basis to
reduce the hematocrit levels.
References
1. Janice L.Hinkle, Kerry H.Cheever (2018) Brunner and
Suddarth’s Text Book of Medical Surgical Nursing ,14th
Edition, South Asian edition, vol.II,Wolters Kluwer.pg no. 925-
938
2. Lewis, S.L.Heitkemper, M.Dirksen, S.R. et al. (2016). Medical
surgical nursing assessment and management of
clinicalproblems.2nd South Asian edition, Mosby Elsevier pg
no.1085- 1098
3. Lizy Sonia & Shaina Sharma (2016).Medical Surgical Nursing-
prep Manual for Undergraduates, vol.I, Thomson Press, India,
Elsevier Publishers. pg no.521

POLYCYTHEMIA

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Learning objectives

By the end of the class , the students will be able to

 Define the term polycythemia

 Enumerate the etiology

 Classify the types of polycythemia

 Enlist the clinical manifestations

 Mention the diagnostic tests

 Describe the management

 Narrate the complications

 Anatomy and physiology of RBC

 Hematology is the study of the blood forming tissues.

 In fetal life ,various tissues produce red blood cells but

Hematopoiesis (red blood cell production) occurs in the

Polycythemia is an increase in hematocrit,

• Polycythemia vera is a rare disease

• The peak incidence of PV is age 50-70 years

– However, it occurs in persons of all age groups,

• Long-term cigarette smoking

• Familial and genetic predisposition

• Primary Polycythemia occurs when excess red blood

• Secondary polycythemia is usually due to increased

• Relative erythrocytosis is an increase in RBC numbers

• Stress polycythemia is a term applied to a chronic (long

Symptoms results from increased blood volume:

• Reddened face with engorged retinal veins

• Itching after bath

• Tinnitus (ringing or buzzing in the ears.)

Symptoms from Increased Viscosity:

• Splenomegaly and hepatomegaly

• Formation of blood clots in the blood vessels

History Collection And Physical Examination:

• Living at high altitudes,

The blood studies will show

• Very low levels of erythropoietin

• PV should be suspected when hemoglobin and/or

BONE MARROW ASPIRATION OR BIOPSY

If an examination of the bone marrow shows that it's

• Men > or = to 36 mL/kg

• Women > or = to 32 mL/kg

– Arterial 02 saturation > or = to 92%

– Leukocytosis with WBC > 12,000/mL

– Increased leukocyte alkaline phosphatase LAP >

• A diagnosis of polycythemia vera is made when a

– any two major and any two minor criteria

The doctor may recommend that to take a low dose

Medication To Decrease Blood Cells

The use of myelosuppressive agents such as

• Pipobroman (vercyte), and

• Hydroxyurea (hydrea) in conjunction with phlebotomy has

Therapy To Reduce Itching.

If you have bother some itching, the doctor may

Splenectomy in patients with painful splenomegaly

or repeated episodes of thrombosis causing splenic

Lifestyle And Home Remedies

Avoid Tobacco.- Using tobacco can cause the blood

Watch for sores.- Poor circulation can make it difficult for

Avoid Extreme Temperatures.

• Monitor for peripheral and cerebral thrombosis.

• Assist the patient for ambulation

• Advice to do regular exercise