Introduction
Primary Myelofibrosis (PMF) is a rare chronic blood disorder that belongs to the group of myeloproliferative diseases. It is characterized by the abnormal proliferation of hematopoietic stem cells without the BCR-ABL1 translocation. This leads to organ enlargement, anemia, low platelet counts, and the presence of immature blood cells. In Israel, approximately 50 to 100 new cases of primary myelofibrosis are diagnosed each year.
At our multidisciplinary hematology clinic in central Tel Aviv, we provide advanced diagnosis and treatment options for patients with primary myelofibrosis. Our experienced team of specialists offers comprehensive and personalized care to improve the quality of life and health outcomes for patients across Israel.
Who is Affected by Myelofibrosis?
Primary myelofibrosis is typically diagnosed in individuals in their 60s and 70s. It is uncommon in patients under the age of 50 and is extremely rare in children.
Symptoms of Primary Myelofibrosis
Many patients experience severe fatigue, which affects two-thirds of those diagnosed. Other common symptoms include:
- Enlarged spleen (splenomegaly)
- Loss of appetite and weight loss
- Low-grade fever, bone pain, night sweats, and itching
- Liver enlargement and portal hypertension in some cases
- Extramedullary hematopoiesis, causing organ enlargement
- Anemia, low platelet counts, and abnormal blood counts
Approximately one-third of patients may have no symptoms, and the disease is discovered during routine blood tests or imaging.
Diagnosis and Risk Assessment
Diagnosis is based on blood tests, bone marrow biopsy, and imaging. At Tel Aviv Medical Center, we use advanced diagnostic tools to assess disease severity and risk. The DIPSS (Dynamic International Prognostic Scoring System) and DIPSS Plus, which includes cytogenetic information, help classify patients into risk categories.
Treatment Options at Our Hematology Clinic in Tel Aviv
Low-Risk Disease Treatment
Patients with low-risk myelofibrosis are monitored regularly. When symptoms develop, the goal of treatment is to improve quality of life. Common treatment options include:
- Hydroxyurea (Hydrea): Reduces spleen size and systemic symptoms.
- Splenectomy: Considered in severe cases when the spleen causes significant symptoms.
- Splenic irradiation: A temporary option for patients who cannot undergo surgery.
Bone marrow transplantation is not recommended for low-risk patients, as it increases mortality rates compared to palliative care.
Intermediate-to-High-Risk Disease Treatment
Patients with intermediate-to-high-risk myelofibrosis have an average life expectancy of fewer than three years. Treatment options include:
- Allogeneic stem cell transplantation: The only curative treatment, but suitable only for younger, healthier patients.
- Reduced-intensity conditioning protocols: Often used in patients over 45.
Factors that improve transplant success include related donors, age under 55, and splenectomy.
Non-Transplant Treatment
For patients who are not candidates for transplantation, the JAK2 inhibitor Ruxolitinib (Jakavi) is an effective option. Benefits include:
- Reduction in spleen size
- Improvement in systemic symptoms
- Improvement in anemia
- Reduction in mortality rates over time
Transformation to Acute Leukemia
Around 10–20% of patients with primary myelofibrosis may progress to acute leukemia within ten years of diagnosis. Unfortunately, treatment options at this stage are limited, and prognosis is poor.
New Treatments and Research in Israel
Clinical trials in Israel are exploring new medications, including Imetelstat, a telomerase inhibitor. Early results have shown disease regression in some patients. Our hematology clinic in Tel Aviv provides access to the latest clinical trials and experimental treatments.
Why Choose Tel Aviv Medical Center?
- Multidisciplinary hematology care in central Israel
- Experienced specialists trained in Israel and abroad
- Access to advanced diagnostic and treatment options
- Personalized care plans tailored to each patient
- Participation in cutting-edge clinical trials
Contact Us
If you or someone you care about has been diagnosed with primary myelofibrosis, contact our clinic in Tel Aviv today. We are here to provide compassionate, expert care and guide you through the best treatment options available in Israel.
Schedule a consultation and receive world-class hematology care in the heart of Tel Aviv.
