What is the disease and how does it affect lifestyle?
Polycythemia vera (PV) is a rare chronic disease of the bone marrow in which too many red blood cells (erythrocytes) are produced, and sometimes too many white blood cells (leukocytes) and platelets (myeloproliferative disease). This leads to increased blood viscosity. This makes it difficult for it to move in small blood vessels and increases the risk of thrombosis.
Patients with PV have a good quality of life. What can affect it are, on the one hand, the patient's complaints of fatigue, headache, itching on the body, etc. They are not mandatory and can be of varying severity. On the other hand, there is the need for monitoring, changes in certain habits, such as smoking. Smokers may have higher hemoglobin without having PV. In that case, we are talking about secondary erythremia.
What are the risk factors for developing PV? How do you assess and mitigate cardiovascular risk factors in PV?
The main risk factors are age over 60 years, mutations in the JAK2 gene (found in over 95% of patients), family history, smoking, and obesity.
There are 2 factors that place patients with proven PV in the group with a high risk of thrombosis. These are age over 60 years and a history of thrombosis in the past. Cardiovascular risk factors, high neutrophil count and JAK2 mutation are also important.
To reduce cardiovascular risk, it is recommended to control blood pressure, cholesterol and blood sugar. Lifestyle adjustments. Monitoring hematocrit levels are extremely important. Taking an antiplatelet (a drug that reduces platelet clumping) is recommended.
What are the first symptoms that give a clear signal that we need to see a doctor?
Clinical manifestations usually begin unnoticed and are associated with increased blood viscosity, which is a result of the increased amount of cells in the blood.
The complaints are most often non-specific. They can be: headache, dizziness, blurred vision, fatigue, difficulty breathing during physical exertion, facial redness, itching after a warm shower. There may be a feeling of heaviness in the left side of the abdomen if the spleen is enlarged.
What is sufficient as a first step is a blood test.
What are the main tips you give when consulting newly diagnosed patients?
It is very important that the patient is informed about their disease. They should know that this is a chronic disease that can be controlled.
Lifestyle adjustments are recommended - exercise, weight loss if excessive, adequate fluid intake, reduction or cessation of smoking, blood pressure control.
The patient should be monitored by a hematologist. The need and benefit of taking the prescribed medications should be explained to them.
What stages does the diagnosis of Polycythemia Vera go through and how long does it all take? What difficulties may arise in diagnosing PV and how does this affect therapeutic strategies?
Diagnosis includes blood count, genetic test for JAK2 mutation, erythropoietin level. Bone marrow examination is not mandatory and is done only at the discretion of the hematologist.
The process can take from several days to 2-3 weeks, depending on laboratory deadlines. Additional tests are also needed to distinguish it from secondary erythremia, which can be the result of, for example, lung, heart diseases, etc.
If patients are not treated, what complications may occur?
In the absence of adequate monitoring and treatment, there is a risk of thrombosis. In high-risk patients, the risk of thrombosis is about 40%. Venous thrombosis is mostly deep vein thrombosis, DVT. Arterial thrombosis has a clinical picture of heart attack, stroke. There is a small risk of bleeding.
After 10-15 years of disease duration, there is a risk of progression to secondary myelofibrosis.
What are the prognoses for newly diagnosed patients these days? Is the disease a fatal diagnosis?
Polycythemia vera is a chronic disease. With good control of hematocrit levels and thrombotic risk, life expectancy can be comparable to that of the general population.
What is the progress in the effectiveness of treatment and what are the latest recommendations for drug therapy?
The goal of treatment is to reduce the risk of thrombosis. The backbone of treatment in most patients is phlebotomy and low-dose antiplatelet agents, in the absence of contraindications. Phlebotomy (bloodletting) is a method of reducing the hematocrit level, which is well tolerated and accessible to patients. In high-risk patients, cytoreductive therapy is also initiated. Those who do not achieve the desired effect or are intolerant are indicated for treatment with JAK inhibitors.
To what extent can the patient's lifestyle be restored after starting therapy?
Most patients lead a normal, active life after starting therapy.
With hematocrit control and no complications, physical and social activity are not significantly affected.
It is only important to follow the recommendations for regular control examinations and an adequate regimen.
Is a complete cure possible and what are the recommendations after that?
The goal of treatment is to control the disease, reduce the risk of complications and maintain quality of life. A complete cure is not possible for now. The patient may have good laboratory parameters, no complaints and no need for phlebotomies and/or cytoreductive treatment. Periodic follow-up is mandatory.
Dr. Vera Stoeva is a physician specializing in clinical hematology and has professional experience in the diagnosis and treatment of hematological diseases. She graduated from the Faculty of Medicine at the Medical University - Sofia in 2009.
In 2016, she acquired a specialty in clinical hematology at the State University of Health Sciences - Sofia. Later, in 2021, she also graduated with a specialty in “Health Management“ at the Southwestern University - Blagoevgrad. In 2024, she defended her scientific and educational degree “Doctor“, and since 2019 she has been an assistant at the State University of Health Sciences - Blagoevgrad. Sofia.
Her professional path began in 2009 as a doctor at Avicena-II-GPPMP, where she worked until 2010. Then she continued her development as a specialist doctor at SBALHZ – Sofia in the period 2010-2016. Since 2017 she has been a specialist doctor at the same medical institution.
Dr. Stoeva is actively engaged in scientific activities. She is a member of the Bulgarian Medical Association in Hematology and the Bulgarian Medical Union. She participates in a number of Bulgarian and international scientific and educational forums, as well as in clinical studies aimed at myeloproliferative and lymphoproliferative diseases.