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Bone Marrow Fibrosis in Hematology

Histologic grading of reticulin and collagen fibrosis with clinical correlations

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

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 Bone Marrow Fibrosis in Hematology?

Bone marrow fibrosis represents abnormal deposition of reticulin and later collagen fibers within the marrow space. Grading uses the European consensus scale from grade zero with normal reticulin to grade three with diffuse coarse reticulin or collagen and osteosclerosis.

Primary myelofibrosis and post polycythemia vera or post essential thrombocythemia myelofibrosis are classical causes. Secondary fibrosis occurs in metastatic carcinoma, granulomatous disease, infections, autoimmune conditions, and toxic exposures. Fibrosis is graded on biopsy with reticulin silver and trichrome stains.

Higher fibrosis grade correlates with leukoerythroblastic blood films, splenomegaly, constitutional symptoms, and shorter survival in primary myelofibrosis. Janus kinase inhibitors such as ruxolitinib reduce splenomegaly and symptoms but variably reverse fibrosis. Allogeneic transplant remains the curative option in eligible high risk patients.

Symptoms

Fatigue and weight loss
Splenomegaly with abdominal fullness
Bone pain in osteosclerotic stages
Anemia and leukoerythroblastic smear
Fever and night sweats

Risk Factors

Prior polycythemia vera or essential thrombocythemia
JAK two CALR or MPL driver mutations
Older age above sixty
Exposure to ionizing radiation or benzene
Metastatic carcinoma to marrow

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • When unexplained leukoerythroblastic film is found
  • When splenomegaly accompanies cytopenia
  • When constitutional symptoms persist
  • When myeloproliferative neoplasm progresses
  • When transfusion need rises

Treatment Methods

01
Bone marrow biopsy with reticulin and trichrome stains
02
Driver mutation testing for JAK two CALR and MPL
03
Risk stratification with DIPSS or MIPSS scores
04
Janus kinase inhibitors for splenomegaly and symptoms
05
Allogeneic transplant in eligible high risk patients
06
Supportive care with transfusion and growth factors
07
Clinical trial enrollment for novel agents

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.

Learn About Hematoloji Department

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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.