DISEASE SCANNER
Global Incurable Diseases Tracker
Myelofibrosis
A rare myeloproliferative neoplasm where scar tissue forms in the bone marrow, disrupting normal blood cell production. Can be primary or secondary to polycythemia vera or essential thrombocythemia. Risk of progression to acute leukemia.
180.0K
15
Symptoms
Treatment Options
Risk Factors
Diagnostic Methods
- 1Complete blood count
- 2Peripheral smear
- 3Bone marrow biopsy
- 4Coagulation studies
- 5Hemoglobin electrophoresis
- 6Flow cytometry
- 7Genetic testing
Prognosis
Median survival 3.5-5.5 years for primary myelofibrosis. Dynamic International Prognostic Scoring System (DIPSS) predicts outcome. Low risk: median 15.4 years; high risk: median 1.3 years. JAK inhibitors (ruxolitinib) reduce symptoms and spleen size but modest survival benefit. Allogeneic stem cell transplant is only curative option with 30-50% survival at 5 years. Transformation to acute leukemia occurs in 10-20% with very poor prognosis. Anemia is common and challenging to manage.
Prevention
- Genetic counseling
- Nutritional adequacy
- Avoidance of triggers
- Vaccinations
- Regular monitoring
- Family screening
Research Status
JAK inhibitors (ruxolitinib, fedratinib) improve symptoms and survival. Allogeneic stem cell transplant only curative. Splenectomy for massive splenomegaly. Supportive care with transfusions.
Affected Countries
Sources
- https://www.hematology.org
- https://medlineplus.gov/blooddiseases.html
- https://www.hematology.org/education/patients
- https://www.ncbi.nlm.nih.gov/books
- https://rarediseases.org/rare-diseases
Medical Disclaimer
This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.