Loading…
Loading…
Primary myelofibrosis is a myeloproliferative neoplasm with marrow fibrosis, splenomegaly and progressive cytopenias.
Quick Answer
Primary myelofibrosis is a myeloproliferative neoplasm with marrow fibrosis, splenomegaly and progressive cytopenias. Dr. Sachita Baniya (NMC 20158, 8+ years of clinical experience in haematology and haemato-oncology) evaluates primary myelofibrosis at Kathmandu Neurology Clinic & Cognitive Center, 117 Lal Durbar Marg, Kathmandu, Bagmati Province 44600, Nepal — evidence-based, medically reviewed.
Myelofibrosis causes marrow scarring, ineffective blood production and enlargement of the spleen. It may arise de novo or from another MPN. Symptoms include fatigue, abdominal fullness, night sweats and weight loss. Treatment focuses on symptom control, anaemia and splenomegaly.
Diagnosis uses blood counts, marrow morphology and fibrosis assessment, mutation testing and spleen assessment. Risk scores guide treatment and transplant decisions.
JAK inhibitors reduce spleen size and symptoms. Supportive care includes transfusion, growth factors and infection prevention. Allogeneic transplant is considered in selected high-risk or fit patients.
Management is individualized and discussed with benefits, limitations, and follow-up.
There is no proven prevention. Early MPN diagnosis and monitoring may allow timely management of progression.
Red flags — seek urgent care
Allogeneic transplant is the only potentially curative option. Other treatments aim to control symptoms, spleen size and blood counts.
They reduce spleen size and constitutional symptoms but do not cure the disease. They require monitoring for cytopenias and infection.
Blood production shifts to the spleen, causing splenomegaly. This can cause abdominal discomfort, early fullness and pain.
Yes. It can progress to acute myeloid leukemia in a proportion of patients. Regular monitoring is important.
No. Transplant is considered for selected fit patients with high-risk disease. Age, comorbidity and donor availability matter.
Transfusion, growth factors, infection prevention, nutrition and symptom control are important components of care.
It confirms fibrosis, assesses cellularity and blasts, and helps distinguish myelofibrosis from other marrow disorders.
Regular blood counts, spleen assessment, symptom review and monitoring for progression or treatment toxicity.
Specialist assessment for abnormal blood counts, anaemia, low platelets, bleeding, clotting and suspected blood disorders.
Care for polycythaemia vera, essential thrombocythaemia and primary myelofibrosis.
Donor stem cell transplant for selected high-risk blood cancers and marrow failure.
Myelodysplastic syndromes are clonal marrow disorders causing low blood counts and a variable risk of progression to leukemia.
Polycythaemia vera is a myeloproliferative neoplasm causing excess red cell production and an increased risk of thrombosis.
Essential thrombocythaemia is a myeloproliferative neoplasm causing persistently high platelets and a risk of thrombosis or bleeding.
Content on this website is for general educational purposes only and does not constitute medical advice, diagnosis, or treatment. It is not a substitute for evaluation by a qualified healthcare professional. Always seek the advice of your physician or other qualified provider with any questions regarding a medical condition. Never disregard professional medical advice or delay seeking it because of something you have read on this website. In case of a medical emergency, contact emergency services immediately. No doctor–patient relationship is established by use of this site or by contacting the clinic through the site.
Reviewed by Dr. Jitendra Prasad Yadav • Last reviewed: 2026-11-10
Content is educational and aligns with standard medical references; individual evaluation may vary. External links provide context and do not imply endorsement.
Doctor — medically reviewed
Dr. Sachita Baniya
Consultant Haemato-Oncologist & Clinical Hematologist • MBBS, FCPS (Clinical Haematology) • NMC 20158
This condition guide was medically reviewed by Dr. Sachita Baniya.
On this site
Article information
Emergency
For sudden neurologic change, thunderclap headache, or seizure >5 min, seek emergency services now.