Myelofibrosis natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

The development of myelofibrosis is a a slow process and it does not cause early symptoms. A significant proportion of the patients can be asymptomatic and the diagnosis is usually made in the setting of an unrelated condition. The most overlapping and common findings encountered are anemia and splenomegaly presenting as weakness, easy fatigability, palpitations, and dyspnea in the case of anemia and early satiety with possible accompanying left upper quadrant discomfort if splenomegaly is present.

The disease has a progressive course and can result in pancytopenia as the bone marrow failure ensues. This can result in bleeding complications, easy bruising, increase in the susceptibility to infections, and worsening anemia. The bone marrow failure paves the way for extramedullary hematopoiesis which mainly occurs in the reticuloendothelial tissues.

If left untreated, myelofibrosis can lead to severe complications, the most feared of which are acute leukemia, heart failure, and portal hypertension.

Natural History

  • Myelofibrosis is a chronic, malignant hematologic disorder which can have a slow progressive course.[1]
  • Progression of the disease can vary from patient to patient and a significant proportion of patients can be asymptomatic.[2][3][4]
  • The disease is characterized by irregularity in the blood cells as a result of marrow fibrosis and the clinical course correlates with this accordingly.[5][6][7][8][9][10][11]
  • Myelofibrosis can manifest as anemia if the pathology involves the red blood cells (RBCs) as the initiating event and it can present as shortness of breath, tiredness, lightheadedness, weakness, headaches, irritability, and pale skin color.[12][13][14]
  • Patients can present with increased susceptibility to infections which can be the presenting or an additional symptom. These infections can be viral, bacterial, or fungal in nature.[15]
  • The distorted platelet production results in bleeding complications such as easy bruising following minimal injury and bleeding from the mucous membranes.[16][17]
  • As a compensation for the lack of efficient blood cell production in the bone marrow, extramedullary hematopoiesis can ensue which will present as splenomegaly, hepatomegaly, lymph node enlargement, and skin, kidney, or lung pathology.[18][19][20][21][22][23][24][22][21][23][25][26][22]

Complications

Common complications of myelofibrosis include:[27][28][29][30][31]

Prognosis

  • Prognosis is generally poor and the median survival for myelofibrosis is 3.5 years to 5.5 years, but patients younger than 55 years have a median survival of 11 years.[30]
  • Poor prognostic factors for myelofibrosis include:[30]
  • Patients without any of the adverse features, excluding age, have a median survival of more than 10 to 15 years, but the presence of any two of the adverse features lowers the median survival to less than 4 years.
  • Karyotype abnormalities can also affect prognosis of myelofibrosis. In a retrospective series, the 13q and 20q deletions and trisomy 9 correlated with improved survival and no leukemia transformation in comparison with the worse prognosis with trisomy 8, complex karyotype, -7/7q-, i(17q), inv(3), -5/5q-, 12p-, or 11q23 rearrangement.[30]

References

  1. ↑ Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
  2. ↑ O'Sullivan JM, Harrison CN (February 2018). "Myelofibrosis: clinicopathologic features, prognosis, and management". Clin Adv Hematol Oncol. 16 (2): 121–131. PMID 29741513.
  3. ↑ Tefferi A (December 2016). "Primary myelofibrosis: 2017 update on diagnosis, risk-stratification, and management". Am. J. Hematol. 91 (12): 1262–1271. doi:10.1002/ajh.24592. PMID 27870387.
  4. ↑ Cervantes F, Pereira A, Esteve J, Cobo F, Rozman C, Montserrat E (November 1997). "[Idiopathic myelofibrosis: initial features, evolutive patterns and survival in a series of 106 patients]". Med Clin (Barc) (in Spanish; Castilian). 109 (17): 651–5. PMID 9488952.
  5. ↑ Bedekovics J, Méhes G (March 2014). "[Pathomechanism and clinical impact of myelofibrosis in neoplastic diseases of the bone marrow]". Orv Hetil (in Hungarian). 155 (10): 367–75. doi:10.1556/OH.2014.29823. PMID 24583557.
  6. ↑ Le Bousse-Kerdilès MC, Martyré MC (October 1999). "Dual implication of fibrogenic cytokines in the pathogenesis of fibrosis and myeloproliferation in myeloid metaplasia with myelofibrosis". Ann. Hematol. 78 (10): 437–44. PMID 10550553.
  7. ↑ Kuter DJ, Bain B, Mufti G, Bagg A, Hasserjian RP (November 2007). "Bone marrow fibrosis: pathophysiology and clinical significance of increased bone marrow stromal fibres". Br. J. Haematol. 139 (3): 351–62. doi:10.1111/j.1365-2141.2007.06807.x. PMID 17910625.
  8. ↑ Reilly JT, Barnett D, Dolan G, Forrest P, Eastham J, Smith A (January 1993). "Characterization of an acute micromegakaryocytic leukaemia: evidence for the pathogenesis of myelofibrosis". Br. J. Haematol. 83 (1): 58–62. PMID 8435338.
  9. ↑ Schmitt A, Drouin A, Massé JM, Guichard J, Shagraoui H, Cramer EM (April 2002). "Polymorphonuclear neutrophil and megakaryocyte mutual involvement in myelofibrosis pathogenesis". Leuk. Lymphoma. 43 (4): 719–24. doi:10.1080/10428190290016809. PMID 12153156.
  10. ↑ Schmitt A, Jouault H, Guichard J, Wendling F, Drouin A, Cramer EM (August 2000). "Pathologic interaction between megakaryocytes and polymorphonuclear leukocytes in myelofibrosis". Blood. 96 (4): 1342–7. PMID 10942376.
  11. ↑ Zahr AA, Salama ME, Carreau N, Tremblay D, Verstovsek S, Mesa R, Hoffman R, Mascarenhas J (June 2016). "Bone marrow fibrosis in myelofibrosis: pathogenesis, prognosis and targeted strategies". Haematologica. 101 (6): 660–71. doi:10.3324/haematol.2015.141283. PMC 5013940. PMID 27252511.
  12. ↑ Birgegard G, Samuelsson J, Ahlstrand E, Ejerblad E, Enevold C, Ghanima W, Hasselbalch H, Nielsen CH, Knutsen H, Pedersen OB, Sørensen A, Andreasson B (November 2018). "Inflammatory functional iron deficiency common in myelofibrosis, contributes to anaemia and impairs quality of life. From the Nordic MPN study Group". Eur. J. Haematol. doi:10.1111/ejh.13198. PMID 30472746.
  13. ↑ Chahdi H, Oukabli M (2018). "[A special form of pancytopenia]". Pan Afr Med J (in French). 29: 209. doi:10.11604/pamj.2018.29.209.14055. PMC 6080970. PMID 30100963.
  14. ↑ Tefferi A (December 2018). "Primary myelofibrosis: 2019 update on diagnosis, risk-stratification and management". Am. J. Hematol. 93 (12): 1551–1560. doi:10.1002/ajh.25230. PMID 30039550.
  15. ↑ Karigane D, Kikuchi T, Sakurai M, Kato J, Yamane Y, Hashida R, Abe R, Hatano M, Hasegawa N, Wakayama M, Shibuya K, Okamoto S, Mori T (July 2018). "Invasive hepatic mucormycosis: A case report and review of the literature". J. Infect. Chemother. doi:10.1016/j.jiac.2018.06.013. PMID 30057341.
  16. ↑ Finazzi G, Vannucchi AM, Barbui T (November 2018). "Prefibrotic myelofibrosis: treatment algorithm 2018". Blood Cancer J. 8 (11): 104. doi:10.1038/s41408-018-0142-z. PMC 6221891. PMID 30405096.
  17. ↑ Hofmann I, Geer MJ, Vögtle T, Crispin A, Campagna DR, Barr A, Calicchio ML, Heising S, van Geffen JP, Kuijpers M, Heemskerk J, Eble JA, Schmitz-Abe K, Obeng EA, Douglas M, Freson K, Pondarré C, Favier R, Jarvis GE, Markianos K, Turro E, Ouwehand WH, Mazharian A, Fleming MD, Senis YA (September 2018). "Congenital macrothrombocytopenia with focal myelofibrosis due to mutations in human G6b-B is rescued in humanized mice". Blood. 132 (13): 1399–1412. doi:10.1182/blood-2017-08-802769. PMID 29898956. Vancouver style error: initials (help)
  18. ↑ Pizzi M, Gergis U, Chaviano F, Orazi A (September 2016). "The effects of hematopoietic stem cell transplant on splenic extramedullary hematopoiesis in patients with myeloproliferative neoplasm-associated myelofibrosis". Hematol Oncol Stem Cell Ther. 9 (3): 96–104. doi:10.1016/j.hemonc.2016.07.002. PMID 27521149.
  19. ↑ Mohyuddin GR, Yacoub A (2016). "Primary Myelofibrosis Presenting as Extramedullary Hematopoiesis in a Transplanted Liver Graft: Case Report and Review of the Literature". Case Rep Hematol. 2016: 9515404. doi:10.1155/2016/9515404. PMC 4739215. PMID 26885416.
  20. ↑ Henry M, Chitlur M, Rajpurkar M, Mastropietro CW, Poulik J, Ravindranath Y (May 2014). "Myelofibrosis, hepatic extramedullary hematopoiesis and ascites associated with vitamin D deficiency in early infancy". J. Pediatr. Hematol. Oncol. 36 (4): 319–21. doi:10.1097/MPH.0b013e31828e548a. PMID 23619118.
  21. ↑ 21.0 21.1 Imai K, Aoi T, Kitai H, Endo N, Fujino M, Ichida S (November 2017). "A case of perirenal extramedullary hematopoiesis in a patient with primary myelofibrosis". CEN Case Rep. 6 (2): 194–199. doi:10.1007/s13730-017-0274-1. PMC 5694411. PMID 28895103.
  22. ↑ 22.0 22.1 22.2 Kwak HS, Lee JM (August 2000). "CT findings of extramedullary hematopoiesis in the thorax, liver and kidneys, in a patient with idiopathic myelofibrosis". J. Korean Med. Sci. 15 (4): 460–2. doi:10.3346/jkms.2000.15.4.460. PMC 3054659. PMID 10983698.
  23. ↑ 23.0 23.1 Mak YK, Chan CH, So CC, Chan MK, Chu YC (February 2002). "Idiopathic myelofibrosis with extramedullary haemopoiesis involving the urinary bladder in a Chinese lady". Clin Lab Haematol. 24 (1): 55–9. PMID 11843900.
  24. ↑ Philipponnet C, Ronco P, Aniort J, Kemeny JL, Heng AE (December 2017). "Membranous Nephropathy and Intrarenal Extramedullary Hematopoiesis in a Patient With Myelofibrosis". Am. J. Kidney Dis. 70 (6): 874–877. doi:10.1053/j.ajkd.2017.06.022. PMID 28821362.
  25. ↑ Mizoguchi M, Kawa Y, Minami T, Nakayama H, Mizoguchi H (February 1990). "Cutaneous extramedullary hematopoiesis in myelofibrosis". J. Am. Acad. Dermatol. 22 (2 Pt 2): 351–5. PMID 2406300.
  26. ↑ Yang M, Roarke M (March 2017). "Diffuse pulmonary extramedullary hematopoiesis in myelofibrosis diagnosed with technetium-99m sulfur colloid bone marrow scintigraphy and single photon emission computerized tomography/CT". Am. J. Hematol. 92 (3): 323–324. doi:10.1002/ajh.24616. PMID 27883206.
  27. ↑ Complications of myelofibrosis. US National Library of Medicine 2016. https://www.nlm.nih.gov/medlineplus/ency/article/000531.htm. Accessed on March 7, 2016
  28. ↑ Signs and symptoms of myelofibrosis. Wikipedia 2016. https://en.wikipedia.org/wiki/Myelofibrosis. Accessed on March 7, 2016
  29. ↑ Kelle, Bayram; Yıldız, Fatih; Paydas, Semra; Bagır, Emine Kılıc; Ergin, Melek; Kozanoglu, Erkan (2015). "Coexistence of hypertrophic osteoarthropathy and myelofibrosis". Revista Brasileira de Reumatologia (English Edition). doi:10.1016/j.rbre.2014.11.004. ISSN 2255-5021.
  30. ↑ 30.0 30.1 30.2 30.3 Disease overview of primary myelofibrosis. National cancer institute 2016. http://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#section/_9. Accessed on March 10, 2016
  31. ↑ Complications of primary myelofibrosis. Dr Henry Knipe and Dr Yuranga Weerakkody et al. Radiopaedia 2016. http://radiopaedia.org/articles/myelofibrosis. Accessed on March 10, 2016


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