Advertisement

Autoimmune Myelofibrosis: A Diagnosis by Exclusion

  • Preeti Tripathi
  • Shivangi J. Harankhedkar
  • Hara Prasad Pati
Chapter

Abstract

Myelofibrosis or bone marrow (BM) fibrosis is a common phenomenon occurring with various benign and malignant disorders. Neoplastic causes of BM fibrosis include various myeloproliferative neoplasms (MPNs), acute megakaryocytic leukemia, lymphoid malignancies, and metastasis of various solid malignancies. Non-neoplastic causes of BM fibrosis include chronic infections (e.g., tuberculosis, kala azar), metabolic causes, storage disorders, drug reactions, and autoimmune disorders [1]. Despite varied etiology and pathogenesis, it is the aberrant production of the fibrogenic cytokines, which is the main cause mediating the fibrosis of BM through stimulation of BM fibroblasts [1].

Keywords

Autoimmune Myelofibrosis Systemic lupus erythomatosus Steroids primary myelofibrosis 

Notes

Conflicts of Interest

Nil.

References

  1. 1.
    Kuter DJ, Bain B, Mufti G, Bagg A, Hasserjian RP. Bone marrow fibrosis: pathophysiology and clinical significance of increased bone marrow stromal fibres. Br J Haematol. 2007;139:351–62.  https://doi.org/10.1111/j.1365-2141.2007.06807.x.CrossRefPubMedGoogle Scholar
  2. 2.
    Paquette RL, Meshkinpour A, Rosen PJ. Autoimmune myelofibrosis. A steroid-responsive cause of bone marrow fibrosis associated with systemic lupus erythematosus. Medicine (Baltimore). 1994;73(3):145–52.CrossRefGoogle Scholar
  3. 3.
    Pullarkat V, Bass RD, Gong JZ, Feinstein DI, Brynes RK. Primary autoimmune myelofibrosis: definition of a distinct clinicopathologic syndrome. Am J Hematol. 2003;72(1):8–12.CrossRefGoogle Scholar
  4. 4.
    Chalayer E, Ffrench M, Cathebras P. Bone marrow fibrosis as a feature of systemic lupus erythematosus: a case report and literature review. Springerplus. 2014;3:349.  https://doi.org/10.1186/2193-1801-3-349.CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Vergara-Lluri ME, Piatek CI, Pullarkat V, Siddiqi IN, O’Connell C, Feinstein DI, Brynes RK. Autoimmune myelofibrosis: an update on morphologic features in 29 cases and review of the literature. Hum Pathol. 2014;45(11):2183–91.  https://doi.org/10.1016/j.humpath.2014.07.017.CrossRefPubMedGoogle Scholar
  6. 6.
    Agarwal A, Morrone K, Bartenstein M, Zhao ZJ, Verma A, Goel S. Bone marrow fibrosis in primary myelofibrosis: pathogenic mechanisms and the role of TGF-β. Stem Cell Investig. 2016;3:5.  https://doi.org/10.3978/j.issn.2306-9759.2016.02.03.CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Piatek CI, Vergara-Lluri ME, Pullarkat V, Siddiqi IN, O’Connell C, Brynes RK, Feinstein DI. Autoimmune myelofibrosis: clinical features, course, and outcome. Acta Haematol. 2017;138:129–37.CrossRefGoogle Scholar
  8. 8.
    Piatek CI, Vergara-Lluri ME, Pullarkat V, Siddiqi IN, O’Connell C, Brynes RK, Feinstein DI. Autoimmune myelofibrosis (AIMF)—clinical features, course and outcome. Blood. 2013;122(21):3714.Google Scholar
  9. 9.
    Hua J, Matayoshi S, Uchida T, Inoue M, Hagihara M. Primary autoimmune myelofibrosis with severe thrombocytopenia mimicking immune thrombocytopenia: a case report. Mol Clin Oncol. 2016;5(6):789–91.  https://doi.org/10.3892/mco.2016.1064.CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Santos FPS, Konoplev SN, Lu H, Verstovsek S. Primary autoimmune myelofibrosis in a 36-year old patient presenting with isolated extreme anemia. Leuk Res. 2010;34(1):e35–7.  https://doi.org/10.1016/j.leukres.2009.08.026.CrossRefPubMedGoogle Scholar
  11. 11.
    Abaza Y, Yin CC, Bueso-Ramos CE, et al. Int J Hematol. 2017;105:536–7.  https://doi.org/10.1007/s12185-016-2129-5.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  • Preeti Tripathi
    • 1
  • Shivangi J. Harankhedkar
    • 1
  • Hara Prasad Pati
    • 2
  1. 1.D.M. HematopathologyAll India Institute of Medical SciencesNew DelhiIndia
  2. 2.Department of HematologyAll India Institute of Medical SciencesNew DelhiIndia

Personalised recommendations