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 blood cells as a result of the 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]
- If the
Complications
Common complications of myelofibrosis include:[15][16][17][18][19]
- Acute myelogenous leukemia
- Infections
- Bleeding
- Thrombohemorrhagic events
- Hepatic failure
- Heart failure
- Gout
- Progressive marrow failure
- Hypertrophic osteoarthropathy
- Splenic rupture
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.[18]
- Poor prognostic factors for myelofibrosis include:[18]
- Age 65 years or older
- Anemia (hemoglobin <10 g/dL)
- Constitutional symptoms (fever, night sweats, or weight loss)
- Leukocytosis (white blood cell count >25 × 109/L)
- Circulating blasts of at least 1%
- 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.[18]
References
- ↑ Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.
- ↑ O'Sullivan JM, Harrison CN (February 2018). "Myelofibrosis: clinicopathologic features, prognosis, and management". Clin Adv Hematol Oncol. 16 (2): 121–131. PMID 29741513.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Complications of myelofibrosis. US National Library of Medicine 2016. https://www.nlm.nih.gov/medlineplus/ency/article/000531.htm. Accessed on March 7, 2016
- ↑ Signs and symptoms of myelofibrosis. Wikipedia 2016. https://en.wikipedia.org/wiki/Myelofibrosis. Accessed on March 7, 2016
- ↑ 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.
- ↑ 18.0 18.1 18.2 18.3 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
- ↑ 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