Myelofibrosis
Chronic myeloproliferative neoplasm with bone marrow fibrosis, extramedullary hematopoiesis, splenomegaly, and constitutional symptoms.
This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.
This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Hematoloji department. Book Appointment →
What is Myelofibrosis?
Myelofibrosis (MF) is a chronic myeloproliferative neoplasm (MPN), occurring as primary myelofibrosis (PMF) or secondary to polycythemia vera (post-PV MF) or essential thrombocythemia (post-ET MF). Driver mutations include JAK2 V617F (60%), CALR (20-25%), and MPL (5-10%); about 10% are triple-negative. Disease arises from hematopoietic stem cell clonal proliferation with cytokine-driven reactive bone marrow fibrosis (TGF-beta, FGF, PDGF), megakaryocyte atypia, and progressive marrow failure with extramedullary hematopoiesis (especially splenic and hepatic).
Clinical features include progressive anemia, splenomegaly (often massive), constitutional symptoms (weight loss, night sweats, fatigue), bone pain, pruritus, splenic infarcts, and signs of portal hypertension. Risk of thrombosis is increased; transformation to acute myeloid leukemia occurs in 10-20%. Diagnosis combines peripheral smear (leukoerythroblastic blood with teardrop red cells), bone marrow biopsy with reticulin/collagen fibrosis grading, JAK2/CALR/MPL mutation testing, and exclusion of other MPNs and reactive causes.
Risk stratification (DIPSS, DIPSS-Plus, MIPSS70+ v2.0) integrates age, hemoglobin, white count, blasts, constitutional symptoms, karyotype, and high-molecular-risk mutations (ASXL1, EZH2, IDH1/2, SRSF2). Therapy is risk-adapted: observation in low-risk asymptomatic, JAK inhibitors (ruxolitinib first-line; fedratinib, momelotinib, pacritinib in select scenarios) for symptomatic disease, supportive care for anemia and cytopenias, and allogeneic stem cell transplant in eligible higher-risk patients as the only curative option.
Symptoms
Risk Factors
When to See a Doctor?
If you experience any of the following symptoms, seek medical attention promptly:
- Persistent unexplained anemia or pancytopenia
- Splenomegaly with constitutional symptoms
- Bone pain with night sweats and weight loss
- Known PV or ET with new anemia or splenomegaly
- Pruritus with hematologic abnormalities
- Sudden severe left upper quadrant pain (splenic infarct)
- Rapidly rising blast count (leukemic transformation)
- Bleeding, infection, or thrombosis in known MF
- Pre-transplant assessment in eligible patients
Treatment Methods
Which Department to Visit?
You can visit our Hematoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.
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You can make an appointment with our specialists or contact us for your concerns.
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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.