KING GEORGE’S MEDICAL UNIVERSITY, LUCKNOW,U.P.

KGMU COLLEGE OF NURSING

TOPIC- POLCYTHEMIA

Presented By:
Mr. Vishal Dubey
M.Sc. Nursing 1st Year (2023-2025)
KGMU College Of Nursing
 OBJECTIVES
At the end of the class, students must be able to:
● define the Polycythemia
● elaborate the Polycythemia
● explain the types of Polycythemia
● elaborate the epidemiology of Polycythemia
● explain the pathophysiology of Polycythemia
● enlist the etiology of Polycythemia
● enlist the clinical manifestation of Polycythemia
● elaborate the diagnostic evaluation of Polycythemia
● enlist the complication of Polycythemia
● explain the management of Polycythemia
 INTRODUCTION

Polycythemia or erythrocytosis
● Increase in the absolute red blood cell (RBC) mass in the body.
● Increase in hemoglobin levels, or hematocrit.
● It is considered physiologic for the particular age and gender.
● The standard RBC mass 36 ml/kg in males and 32 ml/kg in
females.
● Normal hemoglobin levels and hematocrit vary depending on
altitude, ethnicity, and country.
 INTRODUCTION

A healthy adult male

• Hemoglobin- 16 g/dL +/- 2 gm/dl
• Hematocrit- 47% +/- 6%

A healthy adult female

• Hemoglobin 13 g/dL +/- 2 gm/dl
• Hematocrit 40% +/- 6%

Polycythemia in newborns is defined as a

• Central venous hematocrit over 65%
 DEFINITION

• Polycythemia refers to an increased volume of RBCs.

• It is a term used when the hematocrit is elevated (to more than

55% in males, more than 50% in females).

• Dehydration (decreased volume of plasma) can cause an elevated

hematocrit, but not typically to the level to be considered

polycythemia.
 TYPES OF POLYCYTHEMIA

1. Spurious Polycythemia
2. True Polycythemia
• Primary polycythemia
• Secondary polycythemia

3. Neonatal Polycythemia
 POLYCYTHEMIA VERA

Primary polycythemia
A proliferative disorder in which the myeloid stem cells seem to
have escaped normal control mechanisms.
• Increased number of red blood cells in the bloodstream.
• Excess white blood cells and blood clotting cells called platelets.
• These extra cells and platelets cause the blood to be thicker than
normal block the flow of blood through arteries and veins.
• Increased risk of deep vein thrombosis.
 POLYCYTHEMIA VERA

• The bone marrow is hypercellular, and the RBC, WBC, and platelet
counts in the peripheral blood are elevated.
• The RBC elevation is predominant; the hematocrit can exceed 60%. This
phase can last for an extended period (10 years or longer)
 POLYCYTHEMIA VERA

• The spleen resumes its embryonic function of

hematopoiesis and enlarges.
• The bone marrow may become fibrotic, with a resultant
inability to produce as many cells (“burnt out” or spent
phase)
• The disease evolves into myeloid metaplasia with
myelofibrosis.
 EPIDEMIOLOGY

The prevalence of polycythemia vera has been estimated to be approximately

22 cases per 100,000 population. It is believed to occur more frequently
among Jewish patients of Eastern European descent than other Europeans
and Asians. Polycythemia vera shows a male preponderance in all races and
ethnicities, with a male-to-female ratio of approximately 2 to 1. The median
age of presentation of PV is 60 years, with patients seldom seen before the
age of 40. Polycythemia due to hemoglobinopathies and congenital cyanotic
heart diseases is likely to be detected in significantly younger patients.
 PATHOPHYSIOLOGY
High EPO Levels
• Cellular hypoxia can occur due to any
cause that triggers the release of
erythropoietin from the renal peritubular
lining capillary cells.
• A small amount of EPO is produced by the
liver as well. EPO, in turn, acts on
erythroid progenitor cells and stimulates
erythropoiesis.
 PATHOPHYSIOLOGY

Low EPO Levels

• The primary defect in nearly 95% of cases of polycythemia vera is an
acquired mutation in exon 14 of the tyrosine kinase JAK2 (V617F).
Mutations have also been described in exon 12 of JAK2.
• These mutations result in a loss of the auto-inhibitory pseudo-kinase
domain of JAK2, resulting in its constitutive activation.
• This constitutive activation results in both hypersensitivity to EPO and
EPO-independent erythroid colony formation.
 ETIOLOGY

1. Spurious Polycythemia
This occurs due to volume contraction rather than an increase in true RBC
mass.
Causes include
• Severe dehydration due to isolated fluid loss: potentially seen in diarrhea
and severe vomiting
• Gaisbock syndrome: Usually seen amongst obese, hypertensive males.
Smoking, excessive alcohol, and use of diuretics are contributory.
 ETIOLOGY

2. True Polycythemia.
Further stratified based on serum erythropoietin (EPO) levels as follows:
Low serum EPO levels (Primary polycythemia)
• Polycythemia vera
• Primary familial and congenital polycythemia
 ETIOLOGY

High serum EPO levels (Secondary polycythemia)

• High altitude
• Respiratory disorders: Chronic obstructive pulmonary disease (COPD),
Pickwickian syndrome, uncontrolled asthma
• Cyanotic heart diseases with right-to-left shunts
• Renal disorders: Renal cysts, kidney cancer, renal artery stenosis, Bartter
syndrome, focal sclerosing glomerulonephritis
 ETIOLOGY
High serum EPO levels (Secondary polycythemia)
• Elevated carboxyhemoglobin: Usually seen in smokers, people working
on cars in closed spaces, or people working in boiler rooms
• Hemoglobinopathies: High-affinity hemoglobins such as Hb Yakima,
methemoglobinemia
• EPO-secreting tumors: sources include hepatomas, uterine leiomyomas,
and cerebellar hemangiomas
• Iatrogenic causes: Including erythropoietin analog administration,
anabolic steroids, and testosterone replacement therapy
 ETIOLOGY

High-affinity hemoglobins (e.g., Hb Yakima [β99Asp→His]) bind

oxygen more readily but deliver less O2 to tissues.
 CLINICAL MANIFESTATIONS

 Ruddy complexion and

 Splenomegaly(enlarged spleen).
 Increased blood volume
• Headache
• Dizziness
• Tinnitus
• Fatigue
• Paresthesias
• Blurred vision
 CLINICAL MANIFESTATIONS
 Increased blood viscosity
• Angina.
• Claudication.
• Dyspnea.
• Thrombophlebitis.
 Atherosclerotic blood vessels.
 Pruritus-Histamine release due to the increased number of basophils.
 Erythromelalgia -A burning sensation in the fingers and toes, may be
reported and is only partially relieved by cooling.
 DIAGNOSTIC EVALUATION
History
 Fatigue, headache, dizziness, transient blurry vision, amaurosis fugax,
transient ischemic attacks (TIAs).
 Pruritus after a warm water shower, particularly over the back.
 Epistaxis, gastrointestinal (GI) bleeding, easy bruising may be forthcoming.
 Peptic ulcer disease commonly coexists, and patients may present with non-
specific abdominal pain. Left hypochondrial pain and early satiety should
raise the suspicion of splenomegaly.
 DIAGNOSTIC EVALUATION
 Left hypochondrial pain and early satiety should raise
the suspicion of splenomegaly.
 Rarely, patients may present with a history of
unexplained thrombotic complications, such as Budd-
Chiari syndrome or digital infarcts.
 smoking, stay at high altitudes, and congenital cardiac
disease.
 Significant family history may be noted in patients with
hemoglobinopathies.
 DIAGNOSTIC EVALUATION
Physical
• Abnormal facial ruddiness may be prominent.
• Cyanosis and clubbing, cyanotic heart disease.
• Nicotine staining of the nails and teeth provides presumptive evidence of
smoking.
• Morbid obesity could raise the possibility of Pickwickian syndrome,
whereas a barrel chest could suggest obstructive lung disease.
• Examining the abdomen may lead to finding a palpable spleen or
eliciting the bruit of renal arterial stenosis in a thin-built individual.
 DIAGNOSTIC EVALUATION
Hemogram
• Hematocrit levels above 49% in males and 48% in females at sea level
are to be considered suggestive of polycythemia vera.
• The leucocyte count is usually between 10,000 to 20,000/microliter and
may show eosinophilia and basophilia.
• Platelet counts may rarely exceed 1,000,000/microliter.
 DIAGNOSTIC EVALUATION

Radioisotope Studies
• Radioisotope studies using chromium-labeled autologous RBC
transfusions accurately determine the true RBC mass and conclusively
exclude spurious polycythemia.
 DIAGNOSTIC EVALUATION
Serum EPO Levels
Low EPO Levels
• JAK2 mutation studies are virtually diagnostic for polycythemia vera
(95% cases).
• Mutations may occur either in exon 14 (more commonly) or in exon 12.
 DIAGNOSTIC EVALUATION
High EPO Levels
• Measurement of arterial oxygen saturation levels using a pulse-oximeter:
low levels would likely indicate a pulmonary or cardiac cause.
• Normal saturation levels could require further evaluation, such as:
• The use of a cooximeter to rule out methemoglobinemia
• Measurement of carboxyhemoglobin levels for smokers
• Measurement of the P50 of Hb to detect high-affinity hemoglobinopathies
• Relevant investigations to detect a possible EPO-secreting tumor
 DIAGNOSTIC EVALUATION

Serum Ferritin, Vitamin B12, and Folate

Levels
• Low serum ferritin and low folate levels have
been associated more with primary
polycythemia.
• Raised vitamin B12 levels, often striking, may
be observed. This occurs due to increased
transcobalamin III secretion by leukocytes.
 DIAGNOSTIC EVALUATION
Assessment of Renal Function
• Renal function abnormalities indicate a higher likelihood of secondary
polycythemia.
• Uric acid levels are often raised due to increased cell proliferation and
subsequent turnover.
Assessment of Hepatic Status
• Liver cirrhosis and inflammatory liver disease have been associated with
secondary polycythemia and increased RBC proliferation.
 DIAGNOSTIC EVALUATION
Ultrasound
• An ultrasound and Doppler study of the abdomen
would help identify a secondary cause.
• In cases of suspected secondary polycythemia,
the utility of additional investigations such as a
chest radiograph, lung function tests, sleep
studies, and an echocardiograph are to be
considered as appropriate.
 COMPLICATIONS
Secondary polycythemia - Hyperviscosity.
Polycythemia vera
• Venous thrombosis.
• CVA (brain attack, stroke)
• Heart attack (MI)
• Bleeding
• Ulcers
• Frank gastrointestinal bleeding.
 MANAGEMENT

Hypouricemic Agents
Agents such as allopurinol and febuxostat may be
required in cases with significant hyperuricemia.
Management of Pruritus
Depending on the severity of pruritus and the clinical
response to therapy, therapeutic modalities available for
symptomatic relief include antihistamines and selective
serotonin reuptake inhibitors (SSRIs).
 MANAGEMENT

Management of Polycythemia Vera in Pregnancy

• The standard therapeutic measures of phlebotomy and low-dose
aspirin are appropriate in most cases.
• Certain high-risk women may require the addition of pegylated
interferon (IFN)-alpha-Activation of the JAK/STAT pathway
 MANAGEMENT
.

Phlebotomy is an important part of therapy and can be formed repeatedly

to keep the hematocrit within normal range.
 This is achieved by removing enough blood (initially 500 mL once or
twice weekly) to deplete the patient’s iron stores, thereby rendering the
patient iron deficient and consequently unable to continue to
manufacture RBCs excessively
 MANAGEMENT

• Avoid iron supplements.

• Elevated uric acid concentration, allopurinol (Zyloprim).
• Ischemic symptoms, dipyridamole (eg, Persantine) is sometimes used.

Suppress marrow function

 Phosphorus (32P) or chemotherapeutic agents (eg, hydroxyurea [Hydrea])
 Increase the risk for leukemia.
• Hydroxyurea - controlled platelet count.
 MANAGEMENT

Ruxolitinib
• The JAK2 inhibitor ruxolitinib is used when patients are intolerant or
unresponsive to hydroxyurea.
• Ruxolitinib - Increased risks of anemia, often dose-limiting, and
thrombocytopenia.
• The standard recommended dose for polycythemia vera is 10 mg twice a
day.
• Dose reduction is required if hemoglobin drops to below 12 gm/dl.
• A fall in hemoglobin to below 8 gm/dl indicates that dosing is to be
temporarily interrupted.
 MANAGEMENT

Anagrelide (Agrylin)
• Inhibits platelet aggregation
• Controlling the thrombocytosis associated with polycythemia vera.
 NURSING MANAGEMENT

• The nurse’s role is primarily that of educator.

• Risk factors for thrombotic complications should be assessed
• patients should be instructed regarding the signs and symptoms of thrombosis.
• Patients with a history of bleeding are usually advised to avoid aspirin and
aspirin-containing medications, because these medications alter platelet
function.
• Patients must be encouraged to stop smoking.
 NURSING MANAGEMENT
• Genetic counseling must be offered to the families of those with
hemoglobinopathies.
• Patients with polycythemia vera must be discouraged from donating blood.
Because this is a myeloproliferative disorder, blood from donors with
polycythemia vera is not considered appropriate for donation in most countries.
• Minimizing alcohol intake should also be emphasized to further diminish any
risk for bleeding. For pruritus,the nurse may recommend bathing in tepid or
cool water, alongwith applications of cocoa butter–based lotions and bath
products.
 SUMMARY

Polycythemia is a condition characterized by an increase in the

number of red blood cells in the body. This thickening of the blood
can lead to a number of complications, including blood clots, heart
attack, and stroke.
Primary polycythemia, also called polycythemia vera (PV), is a rare
blood cancer that causes the bone marrow to produce too many red
blood cells.
 SUMMARY

Secondary polycythemia is a more common condition that occurs in

response to something else going on in the body, such as chronic
lung disease, smoking, or living at high altitude.
Treatment for primary polycythemia may include phlebotomy
(removing blood from the body), medication to reduce the
production of red blood cells, or radiation therapy. Treatment for
secondary polycythemia focuses on treating the underlying
condition.
 CONCLUSION

Polycythemia is a serious condition that can lead to a number of

complications. If you think you may have polycythemia, it is
important to see a doctor for diagnosis and treatment.
 RECAPTUALISATION

1.What are the two Polycythemia?

2.What iare diagnostic evaluation of Polycythemia?
3.Explain Hypouricemic Agents?
 ASSIGNMENT

EXPLAIN THE ROLE OF NURSE IN MANAGING THE

POLYCYTHEMIA VERA.
 BIBLIOGRAPHY

• BT basvathapa, "Textbook of medical surgical nursing" third

edition, jaypee publication page no 765 to 769.
• Brunner and sudharth’s, Textbook of Medical Surgical Nursing,
2015 page no. 868-894.
ENDOCRINE SYSTEM