Treatment Guideline Chart

A clonal myeloproliferative stem cell disorder characterized by reactive bone marrow fibrosis, extramedullary hematopoiesis, and abnormal cytokine expression leading to systemic symptoms.
It belongs to a group of heterogeneous disorders of the hematopoietic system which is collectively known as Philadelphia chromosome-negative myeloproliferative neoplasms (MPN).
May present either as a de novo disorder [primary myelofibrosis (PMF)] or can develop from the transformation of polycythemia vera (PV) and essential thrombocythemia (ET).

Choice of treatment for patients with myelofibrosus is based on the risk score and presence of symptoms

Myelofibrosis References

  1. Alberta Health Services. Clinical care practice guideline: Myelofibrosis. Version 2. Alberta Health Services. Feb 2020.
  2. Barbui T, Tefferi A, Vannucchi AM, et al. Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNet. Leukemia. 2018 May;32(5):1057-1069. doi: 10.1038/s41375-018-0077-1. PMID: 29515238
  3. Barbui T, Thiele J, Gisslinger H, et al. The 2016 WHO classification and diagnostic criteria for myeloproliferative neoplasms: document summary and in-depth discussion. Blood Cancer J. 2018 Feb;8(2):15. doi: 10.1038/s41408-018-0054-y. PMID: 29426921
  4. Harrison CN, McLornan DP. Current treatment algorithm for the management of patients with myelofibro-sis, JAK inhibitors, and beyond. Hematology Am Soc Hematol Educ Program. 2017 Dec;2017(1):489-497. doi: 10.1182/asheducation-2017.1.489. PMID: 29222297
  5. Kröger NM, Deeg JH, Olavarria E, et al. Indication and management of allogeneic stem cell transplantation in primary myelofibrosis: a consensus process by an EBMT/ELN international working group. Leukemia. 2015 Nov;29(11):2126-2133. doi: 10.1038/leu.2015.233. PMID: 26293647
  6. National Comprehensive Cancer Network (NCCN). NCCN clinical practice guidelines in oncology: Myeloproliferative neoplasms. Version 1.2021. NCCN. 13 Apr 2021.
  7. National Comprehensive Cancer Network (NCCN). NCCN clinical practice guidelines in oncology (NCCN guidelines): Myeloproliferative neoplasms. Version 1.2020. NCCN. 21 May 2020.
  8. National Institute for Health and Care Excellence. Ruxolitinib for treating disease-related splenomegaly or symptoms in adults with myelofibrosis. NICE. 23 Mar 2016.
  9. Reilly JT, McMullin MF, Beer PA, et al. Guideline for the diagnosis and management of myelofibrosis. Br J Haematol. 2012 Aug;158(4):453-471. doi: 10.1111/j.1365-2141.2012.09179.x. PMID: 22651893
  10. Reilly JT, McMullin MF, Beer PA, et al. Use of JAK inhibitors in the management of myelofibrosis: a revision of the British Committee for Standards in Haematology Guidelines for investigation and management of myelofibrosis 2012. Br J Haematol. 2014 Nov;167(3):418-420. doi: 10.1111/bjh.12985. PMID: 24961987
  11. Tefferi A. Clinical manifestations and diagnosis of primary myelofibrosis. UpToDate. 13 Aug 2020.
  12. Tefferi A. Management of primary myelofibrosis. UpToDate. 18 May 2020.
  13. Tefferi A. Primary Myelofibrosis: 2019 update on diagnosis, risk-stratification and management. Am J Hematol. 2018 Dec;93(12):1551-1560. doi: 10.1002/ajh.25230. PMID: 30039550
  14. Tefferi A, Cervantes F, Mesa R, et al. Revised response criteria for myelofibrosis: International Working Group-Myeloproliferative Neoplasms Research and Treatment (IWG-MRT) and European LeukemiaNet (ELN) consensus report. Blood. 2013 Aug;122(8):1395-1398. doi: 10.1182/blood-2013-03-488098. PMID: 23838352
  15. Tefferi A, Guglielmelli P, Pardanani A, et al. Myelofibrosis treatment algorithm 2018. Blood Cancer J. 2018 Aug;8(8):72. doi: 10.1038/s41408-018-0109-0. PMID: 30065290
  16. Vannucchi AM, Barbui T, Cervantes F, et al. Philadelphia chromosome-negative chronic myeloproliferative neoplasms: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2015 Sep;26(Suppl 5):v85-v99. doi: 10.1093/annonc/mdv203. PMID: 26242182
  17. National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: Myeloproliferative neoplasms. Version 2.2022. NCCN. Apr 2022.
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