What exactly is Polycythemia Vera?
This is a disorder of the blood where the bone marrow creates too many blood cells that are red. It also can result in the creation of too many of other blood cells type – white blood and platelets cells. But it is the surplus cells of red blood which thicken the blood and creates majority of the problems associated with this condition.
Polycythemia vera is also known as primary polycythemia and normally develops gradually. The individual can be affected by it for several years before noticing any symptoms or signs. Frequently, polycythemia vera will be discovered when having a blood workup performed for another cause.
This is a very rare disease which occurs most often in male than females, and it is rare in individuals under 40 years of age. The exact cause is not known.
With no medical management, polycythemia vera is life-threatening. But, with the appropriate medical care, numerous individuals experience very few problems linked to this illness.
Polycythemia Vera Symptoms
In the initial phases, polycythemia vera normally does not cause any symptoms or signs. But, as this disease progresses, the individual might experience:
- Dizziness
- Headache
- Redness of the skin
- Itchiness, following a shower or warm bath
- Shortness of breath
- Difficulty breathing when lying down
- Numbness, burning, tingling or weakening in hands, arms, feet or legs
- Feeling of bloating or fullness in the upper left abdomen because of enlarged spleen
- Chest pain
- Fatigue
If an individual has any symptoms or signs of polycythemia vera, they should arrange for a visit with the physician for an assessment.
Since polycythemia vera makes the blood thicken as well as slows the flow of blood, it increases the risk of blood clots. When blood clots happen in the head, it may lead to a stroke. Pursue emergency treatment if you notice any below listed symptoms or signs of a possible stroke:
- Weakness, paralysis or numbness of the face, leg or arm which is sudden – normally on one side only of the body
- Difficulty with understanding speech or problems talking which is sudden – aphasia
- Blurred, decreased or double vision which is sudden
- Loss of balance or coordination, dizziness which is sudden
- Severe stark headache or strange headache which is sudden and can go along with a stiff neck, pain in the face, pain in between the eyes, altered consciousness or vomiting
- Difficulties with memory, confusion, spatial awareness or orientation
Polycythemia Vera Causes
Blood in the human body consists of plasma which is liquid and 3 sorts of cells which flow in the plasma:
- Erythrocytes or red blood cells – transfers oxygen from the lungs, thru the bloodstream, to the brain and the body’s other tissues and organs. The body desires a resource of blood which is oxygenated to work properly. Oxygenated blood aids the body in acquiring its get-up-and-go and provides the skin with a hale and hearty glow.
- White blood cells – fight infection
- Platelets – blood cells that help the blood clot after an injury or cut
Typically, the body regulates cautiously the total cells of blood as well as the proportion of one blood cell type to others. However in diseases known as myeloproliferative disorders, of which polycythemia vera is one, the apparatus by which the body manages blood cell production becomes compromised, and the bone marrow creates too few or too much of certain types of blood cells.
Mutation
The difficulty with the production of blood cell linked to polycythemia vera can be triggered by a mutation or change to the DNA in only a solitary cell in the marrow of bone. With polycythemia vera, medical professionals have isolated this mutation and believe that it is a change in a protein switch which tells the cells to grow. Precisely, it is a transmutation in the protein of JAK2 (the JAK2 V617 F mutation). Approximately 90% or more of individuals who have polycythemia vera as well as approximately one/half of those individuals with other conditions of myeloproliferative, have this alteration. Physicians and other medical professionals do not understand fully the character of this transmutation or its associations for the treatment of this disease.
DNA is an instruction set for the cells in the body, telling the cells when and how to divide and grow. The DNA transmutation which is accountable for polycythemia vera causes the production of blood cell to go askew. All the cells which are produced by that first mutant cell have the same problem, and they begin to dominate the production of blood cells.
The DNA transmutation that causes polycythemia vera transpires after conception – denoting that the father and mother do not have it – so it is acquired, instead of genetic from one of the parents. Medical researchers and physicians do not know what exactly causes this mutation.
There are some factors which do increase an individual’s risk of emerging polycythemia vera:
Family history
There are some cases where polycythemia vera seems to be in families, which is an indicator of genetic aspects other than JAK2 contributing to this disease.
Sex
Polycythemia vera affects men more than women.
Age
Accorded by the National Heart, Lung and Blood Institute, polycythemia vera is most likely in those who are older than 60 years of age. It is rare in individuals less than 20 years of age.
Polycythemia Vera Complications
Some complications which are possible with polycythemia vera are:
Blood clots
This illness makes the blood much thicker, which slows the flow of blood thru arteries and veins. Greater thickness of blood and decreased flow of blood, are irregularities in the platelets, increases the threat of blood clots. Clots may cause a heart attack, a stroke, or the obstruction in an artery in the lungs – “pulmonary embolism” – or in a deep vein in a muscle – “deep vein thrombosis”.
Inflated spleen – splenomegaly
The spleen aids the body in fighting infections as well as filtering undesirable material, for example damaged or old blood cells. This increase in the number of blood cells created by polycythemia vera causes the spleen to work harder, which makes it become distended. If the spleen becomes too big, it often may have to be removed.
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Problems with skin
Polycythemia vera can cause the skin to become itchy, particularly following a warm shower or bath, or following a night in a warm bed. Individuals may notice a tingling or burning feeling in the skin, especially on the arms, hands, legs, or feet. The skin can look red, particularly on the face, earlobes and palms.
Problems cause by too many red blood cells
Increased red blood cells often lead to numerous complications, including open wounds on the inside lining of the stomach, upper esophagus or small intestine– peptic ulcers –joint inflammation – gout – and stones made of uric acid in the kidneys.
Other blood illnesses
In very rare circumstances, polycythemia vera can cause other diseases of the blood including a progressive condition where bone marrow is exchanged with scar tissue called “myelofibrosis”, a disorder in which stem cells do not mature or correctly function called “myelodysplastic syndrome” or cancer of the blood and bone marrow called acute leukemia.
Polycythemia Vera Diagnosis and Tests
Blood tests
Physicians most commonly use testing of the blood in order to identify polycythemia vera. Actually, in most circumstances, polycythemia vera is revealed thru bloods tests which are implemented for other causes. If an individual has polycythemia vera, blood tests can disclose:
- Upsurge in the amount of red blood cells – as well as in many cases, an upsurge in white blood cells and platelets.
- Raised levels of hemoglobin – the protein which is iron-rich in red blood cells which carries oxygen.
- Raised measurement of hematocrit – the percent of red blood cells which form the total volume of the blood.
- Inferior levels of oxygen– in the blood
- Very small points of erythropoietin EPO – hormone which motivates bone marrow to create new red blood cells; this extra red blood cells shuts off the creation of EPO by the kidneys.
Bone marrow biopsy or aspiration – If the physician believes that you might have polycythemia vera, he/she can suggest a bone marrow biopsy or aspiration to accumulate a sample of the bone marrow. This bone marrow biopsy comprises extracting a sample of the solid bone marrow material. An aspiration of bone marrow is normally done at the similar time as the biopsy. Thru an aspiration, the physician removes a sample of the fluid portion of the marrow.
If the examination of the marrow displays that it is creating higher than standard numbers of blood cells, it can be a symptom or sign of polycythemia vera. If you do have polycythemia vera, study of the bone marrow or blood also can show the change in DNA (JAK2 V617F mutation) that is linked with the illness.
Polycythemia Vera Treatment
Polycythemia vera is a condition which is chronic that cannot be cured. Treatment is aimed on reducing the amount of blood cells. In the majority of cases, treatment can stop or prevent complications from polycythemia vera and decrease or eliminate the signs and symptoms of this disease.
Treatment may include:
Phlebotomy
Normally the first option of treatment for those with polycythemia vera. This comprises drawing a specific quantity of blood from a vein. This decreases the number of blood cells and drops the blood volume, making it easier for the blood to function properly. How often this is needed rests on the severity of the disorder.
Medication for decreasing blood cells
Phlebotomy by itself cannot always be enough to manage the symptoms, signs and problems of polycythemia vera in certain individuals. If this is the case, the physician may prescribe drugs such as hydroxyurea or anagrelide, to suppress the ability of the bone marrow to create blood cells. Radioactive phosphorus may also be another way to decrease the number of blood cells. Interferon-alpha may be used to stimulate the immune system to fight any overproduction of red blood cells.
Aspirin low-dose
The physician can recommend low dosage of aspirin to decrease the threat of blood clots. Low-dosages of aspirin can as well help decrease the burning type pain in the feet or hands.
Therapy to Reduce Itching
If there is itching which is bothersome, the physician may also recommend medication such as antihistamines or H-2 receptor blockers, as well as recommend ultraviolet light therapy to relieve the discomfort.
Lifestyle/Home Remedies
Exercise
Moderate exercise, for example walking, can improve the flow of blood, which decreases the risk of blood clots. Leg as well as ankle stretches and exercise also can improve blood circulation.
No tobacco
Tobacco use can cause blood vessels to narrow, increasing any risk of stroke or heart attack due to blood clots.
Pamper the skin
To reduce itching, bathe in cool water and pat the skin dry. Avoid heated whirlpools, hot tubs, as well as hot baths or showers. Do not scratch, as that can damage the skin and increase a risk of infections developing. Use lotion to keep skin moisturized.
Extreme temperatures should be avoided
Poor blood flow increases the risk of injury from hot and cold temperatures. During cold weather, always wear warm clothing, especially on the hands and feet. In hot weather, protect the body from the sun as well as drink plenty of liquids.
Watch for sores
Circulation which is poor can make it difficult for sores to heal, especially on the hands and feet. Inspect feet regularly as well as tell the physician about any sores.
