Diagnosis
Myelofibrosis is diagnosed from the bone marrow, which shows fibrosis, atypical megakaryocytes and reduced normal blood production. JAK2, CALR or MPL mutations support a clonal MPN; secondary causes of marrow fibrosis must be excluded.
Blood counts, blast percentage, spleen size, symptoms and cytogenetics are combined in prognostic scoring. The distinction between primary myelofibrosis and progression from PV or ET matters for treatment planning.
Related: Haematology → · Dr. Sachita Baniya profile → · All conditions →
Symptoms and complications
Common problems include fatigue, night sweats, weight loss, itching, bone pain, early satiety from an enlarged spleen, anaemia and bleeding or thrombosis. Portal hypertension and extramedullary disease can occur.
The review looks for signs of progression to accelerated or blast phase, and for complications that need urgent treatment.
- Symptom burden and spleen size
- Anaemia and transfusion need
- Blast count and cytogenetics
- Thrombosis, bleeding and infection
Treatment options
JAK inhibitors can reduce spleen size and constitutional symptoms. Supportive care includes transfusion, erythropoiesis-stimulating agents in selected patients, antibiotics and vaccination. Hydroxyurea may be used for high counts in some situations.
Allogeneic transplant is the only potentially curative option and is considered for selected higher-risk or younger patients. The decision weighs disease risk, donor availability, fitness and patient preference.
Access and booking
Dr. Baniya consults at Kathmandu Neurology Clinic & Cognitive Center, Durbar Marg, and also practises at Kathmandu Cancer Center (KCC) Nepal, Civil Service Hospital, Kathmandu, and Advanced Care Hospital. Appointments are arranged at the Durbar Marg clinic or through the respective hospital or cancer centre. Bring all blood counts, marrow, flow cytometry and molecular reports.