The Pathogenesis of Myelofibrosis in Myeloproliferative Disorders

  1. JEROME E. GROOPMAN, M.D.
  1. Division of Hematology-Oncology, UCLA School of Medicine;
    Los Angeles, California

    Excerpt

    The pathogenesis of bone marrow fibrosis in the myeloproliferative disorders is unknown. Various other conditions, benign and malignant, that involve the marrow, including osteopetrosis, Paget's disease, thorium dioxide administration, hairy cell leukemia, and carcinoma of the breast, may also be associated with marked myelofibrosis. The bone marrow scarring in such diverse disorders probably arises via different mechanisms. My comments here focus on the genesis of marrow fibroblast proliferation in myeloproliferative disorders.

    Fibroblasts are the source of the silver-staining precollagen termed reticulin that replaces normal marrow stroma in disorders with myelofibrosis. Although the bone marrow fibroblast was once believed to be

    Acknowledgments

    The author thanks Drs. M. J. Cline, D. W. Golde, R. I. Handin, and C. D. Scher for critical discussion of the manuscript.

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