Polycythemia| Polycythemia Vera

Polycythemia Rubra Vera  |Polycythemia 

Introduction:

This article discusses different causes of polycythemia and methods employed to diagnose polycythemia. Here, learn about the tests, procedures, indications, and more.

Keywords:  

Polycythemia| Polycythemia vera|Relative polycythemia|Primary polycythemia|CBC test |Asolute|Blood disorder|Myeloprolerative disease|Pluripotent  

Table of contents

1.	Introduction
2.	Classification
3.	Causes
4.	Signs and Symptoms
5.	Diagnosis and Treatment

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Definition

Polycythemia is when the blood's hematocrit value and hemog

lobin content increase above the average levels for the individual's age and sex.

The hematocrit value for adult males is> 49%, and for adult females, it is>47%.

For adult males, hemoglobin level > 16.5 gms/Dl of blood, and in adult females > 16 grams /Dl

In New Nates and Children, definitions and values are different.

Polycythemia is different from erythrocytosis. In erythrocytosis, only the number of erythrocytes increases above the normal range for the individual's age and sex.

Classification

Polycythemia is divided into

1. Relative Polycythemia and

2. Absolute Polycythemia

(a) Primary Polycythemia is also known as Polycythemia rubra vera or simply Polycythemia Vera.

(b)Secondary Polycythemia.

Relative Polycythemia

A decrease in the plasma volume of blood causes relative Polycythemia. Since only plasma volume decreases and blood cells do not decrease, the hematocrit value appears to be raised.

Causes of relative Polycythemia

Any cause that leads to fluid loss—hypovolemia—will cause relative Polycythemia, such as burn, diarrhea, vomiting, excess sweating in hot climates, diuretics, and stress.

Primary Polycythemia

Overproducing red blood cells is due to primary defects in the bone marrow. Pluripotent stem cells or hemopoietic cells are affected. It is a myeloproliferative disease.

This may be congenital or familial and may be acquired late in life.

It is a benign hereditary disease due to an autosomal dominant mutation in the JAK2.

Pluripotent stem cells have receptors for erythropoietin.

When erythropoietin molecules combine with their

receptors, they stimulate Janus Kinase 2 in the pluripotent

stem cells, causing the production of different types of blood

cells.

In primary Polycythemia, there is a Janus Kinase 2 gene mutation. Mutated JAK2 molecules work independently and produce a large number of blood cells. They do not need erythropoietin stimulation for red cell production. All formed elements of blood are raised.

Increased erythropoietin level, a protein secreted mainly from the kidney and, to some extent, by the liver, causes secondary Polycythemia.

Hypoxia is a powerful stimulant of erythropoietin production. Hence, hypoxia from any cause will increase red blood cell count.

Causes of erythropoietin production

1. Chronic low oxygen level.

Image produced by the author

2. High altitude-hypoxia.

3. Some drugs like testosterone and androgenic hormones.

4. Diseases causing hypoxia, for example, cyanotic heart diseases, chronic obstructive pulmonary disease (COPD), sleep apnea, methemoglobinemia, and chronic carbon monoxide poisoning.

5. Certain cancers- like renal cell cancers and hepatic tumors.

6. Heavy smoking.

7. Genetic mutation

8. Abnormalities of hemoglobin oxygen dissociation curve.

9. EExcess blood transfusion

10. Post-transplant erythropoiesis -after renal transplant.

Signs and symptoms

Increasing hematocrit value increases blood viscosity, slowing blood flow in the capillaries and favoring clot formation.

The red complexion, red face, and red palms.

CNS- dizziness, fatigue, light-headedness, syncope, headache. Transient ischemic attack and stroke.

Respiratory system cyanosis and clubbing.

C.V.S. angina, myocardial infarction, congestive heart failure.

Skin pruritic, rash, and itching after hot baths are widespread.

Numbness and tingling sensation in limbs and different parts of the body.

Gout due to excess destruction of red blood cells.

Pain in the upper abdomen due to hepatomegaly and splenomegaly.

Loss of appetite. Weakness, lethargy.

Diagnosis

Family history may be present. Personal history.

High altitude dwellers

CBC -hematocrit value raised.

A somatic mutation in the JAK2 gene is positive.

Erythropoietin level estimation- erythropoietin level is normal or decreased in primary Primary Polycythemia and increased in secondary Polycythemia.

Bone marrow examination.

Treatment

Phlebotomy is the essential treatment.

Treatment of associated conditions, such as gout.

Aspirin.

Lifestyle modification.

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Hashtags: 

Polycythemia # Polycythemia vera# Relative polycythemia# Primary polycythemia#CBC test # Asolute # Blood disorder# Myeloprolerative disease#Pluripotent  

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