Secondary myelofibrosis – Etiology, Pathogenesis, Investigations and Management


Normal bone marrow
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  • Myelofibrosis refers to replacement of normal bone marrow constituents by fibrous tissue
  • Secondary myelofibrosis occurs as a result of various pathological processes that involve the bone marrow

Causes of secondary myelofibrosis:

  • Neoplastic – infiltration by cancer cells (primary is usually located in breast, lung or prostate)
  • Infective – tuberculosis, fungal infections, HIV
  • Metabolic – Gaucher’s disease
  • Hematological malignancies – chronic myeloid leukemia, multiple myeloma, lymphomas
  • Radiation, radiomimetic drugs
  • Others – Sarcoidosis,  congenital osteopetrosis

Pathophysiology:

  • Marrow contents are replaced by fibrous tissue
  • There is extramedullary hematopoiesis in sites like spleen and liver
  • Increased number of circulating hematopoietic progenitor cells

Investigations:

  • Bone marrow aspiration gives a ‘dry tap’ – no cells are obtained
  • Bone marrow biopsy reveals fibrosis of bone marrow
  • Peripheral smear
    • anemia – normocytic, normochromic
    • leukoerythroblastosis – blast forms of RBC’s and WBC’s

Management:

  • Treat the underlying condition
  • Bone marrow transplantation

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