True erythroblastosis (PV) can cause a patient’s bone marrow to produce too many red blood cells. The body needs red blood cells to carry oxygen to all parts of the body, but too many red blood cells can cause the patient’s blood to become sticky and form clots. In some cases, blood clots can trigger a heart attack or stroke.
With effective treatment the patient’s red blood cell count can be lowered, preventing clots from forming. In addition the right treatment can relieve other symptoms such as headaches and vision problems. With proper treatment, patients can maintain their health and improve their quality of life.
Venotomy
This is the main treatment option for true erythrocytosis. Patients are bled to remove excess red blood cells, thereby reducing the number of red blood cells in the body to facilitate near normal blood viscosity.
When a patient is first diagnosed with true erythrocytosis, the doctor may perform a phlebectomy. The procedure is similar to a blood donation, in which a doctor or nurse sticks a needle into a vein in the patient’s arm and drains a small amount of blood from his or her body.
The goal of this treatment is to lower the erythrocyte volume level. Erythrocyte volume is the percentage of red blood cells in the volume of whole blood.
Patients are treated once a week or once a month until the RBC volume drops to about 45%. When the erythrocyte level has decreased, the patient may be treated less frequently with phlebotomy and bloodletting.
After a patient’s red blood cell level has decreased, certain prescription medications may be used to slow the rate of new red blood cell formation in the bone marrow.
Hydroxyurea
Hydroxyurea is an anticancer drug that slows the growth of newborn cells in the body. In true erythrocytosis, it reduces the levels of red blood cells and platelets, where the platelets act to promote blood clotting. If a patient is taking this medication, then a phlebotomy may not be necessary to release blood. Hydroxyurea may also prevent or treat splenomegaly (a complication of true erythrocytosis).
Hydroxyurea is an oral capsule that may cause side effects, including:
- nausea;
- vomiting;
- Constipation;
- Diarrhea;
- Rash.
In extremely rare cases, it may cause cells to become cancerous. Although the risk of this is very low, clinicians will monitor for cancer while patients are taking hydroxyurea.
Interferon-alpha
Interferon-alpha boosts a patient’s immune system function, targeting rapidly dividing red blood cells and slowing down their production of new cells.
Interferon-alpha can be administered by subcutaneous injection, and possible side effects during treatment include:
- flu-like symptoms (fever, chills, and muscle aches);
- Fatigue;
- nausea and vomiting.
Long-acting interferon, also known as pegylated interferon, has relatively few side effects.
Aspirin
Aspirin can prevent blood clots from forming and can also relieve patients of symptoms such as burning pain in the hands and feet, itching, and bone pain. Doctors may recommend that patients take a low-dose aspirin every day.
Taking aspirin every day carries some risk that it may cause an increased risk of bleeding, especially in the stomach or other parts of the digestive system. Thus, patients should talk to their doctor before starting aspirin to fully understand the risks associated with side effects.
Other medications
Doctors will also consider treatment with certain newer drugs, such as anagrelide, leucovorin, imatinib, and ruxolitinib. These drugs help certain types of blood cells function better, resulting in improved flow and decreased viscosity of the patient’s blood.
The new drugs mentioned above may be clinical alternatives if other treatment options are not working or if patients cannot tolerate drugs such as hydroxyurea and interferon. Also, the clinical effects of each drug are different, so doctors will inform patients in detail about the side effects of each type of drug and their latest efficacy assessment results.
Radiotherapy
This therapy slows down the production of red blood cells in the bone marrow, which reduces blood viscosity and improves blood flow in the patient’s body. However, physicians rarely use radiotherapy regimens because it also carries a clinical risk of inducing a hematologic tumor (leukemia).
In addition to following a treatment plan, patients can take the following steps to improve their quality of life:
Exercise daily
Being active helps keep the blood flowing and prevents blood clots from forming. Patients can also perform regular stretching exercises, especially in the legs and ankles, to improve blood flow.
Take cold showers and keep warm
- Cold baths: Avoid skin irritation and pat the skin gently with a washcloth afterwards, without rubbing the skin. Moisturize your skin well or use over-the-counter antihistamines.
- Keep warm: True erythrocytosis affects the patient’s blood flow, which can easily cause the patient to lose heat in the hands and feet. Therefore, when it is very cold outside, patients should remember to wear gloves and socks.
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Drink an adequate amount of fluids
Intake of adequate amounts of fluids will help prevent patients from having overly sticky blood.
Avoid injury and sun exposure
- Trying to avoid trauma to the feet: Patients should wear good shoes to prevent damage to the skin of their feet. You need to keep an eye on your feet on a daily basis to see if they are cut or have ulcers.
- Protect your skin from the sun: Wear sunscreen with an SPF (sun protection factor) ≥ 30 every time you go outside. You also need to wear protective clothing and a wide-brimmed hat.
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Learn to de-escalate
It is also common for patients to have certain complex emotions when they have a more serious illness. When concerns about the disease begin to cause negative emotions such as anxiety and depression, it is necessary for patients to go to their primary care physician or counselor for communication, or to join a patient club or talk to close friends and family so that others can understand what they are going through and be comforted and helped by them.