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
Myelofibrosis has a very indolent course.[1] If left untreated, myelofibrosis may progress to develop acute myelogenous leukemia, thrombohemorrhagic events, and progressive marrow failure. Common complications of myelofibrosis include infections, bleeding, hepatic failure, heart failure, and gout.[2][3][4][5][6] 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.[5]
Natural History
Myelofibrosis has a very indolent course.[1]
Complications
Common complications of myelofibrosis include:[2][3][4][5][6]
- 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.[5]
- Poor prognostic factors for myelofibrosis include:[5]
- 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.[5]
References
- ↑ 1.0 1.1 Diagnosis of myelofibrosis. Wikipedia 2016. https://en.wikipedia.org/wiki/Myelofibrosis. Accessed on March 8, 2016
- ↑ 2.0 2.1 Complications of myelofibrosis. US National Library of Medicine 2016. https://www.nlm.nih.gov/medlineplus/ency/article/000531.htm. Accessed on March 7, 2016
- ↑ 3.0 3.1 Signs and symptoms of myelofibrosis. Wikipedia 2016. https://en.wikipedia.org/wiki/Myelofibrosis. Accessed on March 7, 2016
- ↑ 4.0 4.1 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.
- ↑ 5.0 5.1 5.2 5.3 5.4 5.5 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
- ↑ 6.0 6.1 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