Myelofibrosis overview
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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 is a hematological disorder where there is replacement of bone marrow with collagenous connective tissue and progressive fibrosis, or the replacement of the marrow with scar tissue. It is also classified as a myeloproliferative disorder.[1][2] The term myelofibrosis alone usually refers to primary myelofibrosis (PMF), also known as chronic idiopathic myelofibrosis (CIMF); the terms idiopathic and primary mean that the disease is of unknown or spontaneous origin. This is in contrast with myelofibrosis that develops secondary to polycythemia vera, essential thrombocythaemia, leukemia, or lymphoma (secondary myelofibrosis). Myelofibrosis is a form of myeloid metaplasia, which refers to a change in cell type in the blood-forming tissue of the bone marrow, and often the two terms are used synonymously.[2]
Historical Perspective
Myelofibrosis was first discribed by Gustav Heuck, a German surgeon, in 1879, under the title of 'Two cases of leukemia with peculiar blood and bone marrow findings'.[3]
Classification
Based on the origin, myelofibrosis may be classified into two subtypes: primary and secondary.[4]
Pathophysiology
Myelofibrosis is a clonal neoplastic disorder of hematopoiesis, the formation of blood cellular components.[5] It is one of the myleoproliferative disorders, diseases of the bone marrow in which excess cells are produced. Genes involved in the pathogenesis of myelofibrosis include JAK2, CALR, and MPL.[6]
Causes
Common causes of myelofibrosis include genetic mutations. The genes involved are listed here.[6][7][8]
Differentiating Myelofibrosis from other Diseases
Myelofibrosis must be differentiated from other diseases that cause diffuse bone sclerosis, such as sickle cell disease, hyperthyroidism, sclerosing bone dysplasia, osteoblastic metastases, and Paget's disease.[9][10] Myelofibrosis must be differentiated from other diseases that cause splenomegaly, such as anemia, CML, polycythemia rubra vera, cirrhosis, infections, neoplastic, and lipid storage disorders.[9][11]
Epidemiology and Demographics
The prevalence of myelofibrosis is approximately 1 per 100,000 individuals worldwide. Myelofibrosis is a disease that tends to affect the middle-aged and elderly population. The mean age at diagnosis is 60 years.[12] Males are more commonly affected with myelofibrosis than females. The male to female ratio is approximately 1.5 to 1.[13] Myelofibrosis usually affects individuals of the Ashkenazi Jews race. African American, Latin American, and Asian individuals are less likely to develop myelofibrosis.[14]
Risk Factors
Common risk factors in the development of myelofibrosis may be age, other myeloproliferative disorders, radiation, or industrial chemical exposure.[15]
Natural History, Complications and Prognosis
Myelofibrosis has a very indolent course.[16] 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.[17][18][19][20][21] 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.[20]
Diagnosis
Diagnostic Criteria
According to the World Health Organization (WHO) diagnostic criteria for primary myelofibrosis, polycythemia vera, and essential thrombocythemia, the diagnosis of primary myelofibrosis is made when all three of the following major diagnostic criteria and at least two minor criteria are met.[22][23]
Staging
There is no established system for the staging of myelofibrosis.[20]
References
- ↑ Myelofibrosis. Dr Henry Knipe ◉ and Dr Yuranga Weerakkody et al. Radiopaedia 2016. http://radiopaedia.org/articles/myelofibrosis. Accessed on March 7, 2016
- ↑ 2.0 2.1 Myelofibrosis. Wikipedia 2016. https://en.wikipedia.org/wiki/Myelofibrosis. Accessed on March 7, 2016
- ↑ Tefferi, A (2007). "The history of myeloproliferative disorders: before and after Dameshek". Leukemia. 22 (1): 3–13. doi:10.1038/sj.leu.2404946. ISSN 0887-6924.
- ↑ Classification of myelofibrosis. Dr Henry Knipe and Dr Yuranga Weerakkody et al. Radiopaedia 2016. http://radiopaedia.org/articles/myelofibrosis. Accessed on March 14, 2016
- ↑ Causes of myelofibrosis. Wikipedia 2016. https://en.wikipedia.org/wiki/Myelofibrosis. Accessed on March 7, 2016
- ↑ 6.0 6.1 Tefferi, A; Lasho, T L; Finke, C M; Knudson, R A; Ketterling, R; Hanson, C H; Maffioli, M; Caramazza, D; Passamonti, F; Pardanani, A (2014). "CALR vs JAK2 vs MPL-mutated or triple-negative myelofibrosis: clinical, cytogenetic and molecular comparisons". Leukemia. 28 (7): 1472–1477. doi:10.1038/leu.2014.3. ISSN 0887-6924.
- ↑ Baxter EJ, Scott LM, Campbell PJ; et al. (2005). "Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders". Lancet. 365 (9464): 1054–61. doi:10.1016/S0140-6736(05)71142-9. PMID 15781101.
- ↑ Pikman Y, Lee BH, Mercher T; et al. (2006). "MPLW515L is a novel somatic activating mutation in myelofibrosis with myeloid metaplasia". PLoS Med. 3 (7): e270. doi:10.1371/journal.pmed.0030270. PMC 1502153. PMID 16834459. Unknown parameter
|month=ignored (help) - ↑ 9.0 9.1 Differential diagnosis of myelofibrosis. Dr Henry Knipe and Dr Yuranga Weerakkody et al. Radiopaedia 2016. http://radiopaedia.org/articles/myelofibrosis. Accessed on March 10, 2016
- ↑ Diffuse bony sclerosis: differential diagnosis. Dr Craig Hacking and Dr Yuranga Weerakkody et al. Radiopaedia 2016. http://radiopaedia.org/articles/diffuse-bony-sclerosis-differential-diagnosis. Accessed on March 10, 2016
- ↑ Splenomegaly. Dr Henry Knipe and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/Italic textarticles/splenomegaly. Accessed on March 11, 2016
- ↑ Epidemiology of myelofibrosis. Dr Henry Knipe and Dr Yuranga Weerakkody et al. Radiopaedia 2016. http://radiopaedia.org/articles/myelofibrosis. Accessed on March 8, 2016
- ↑ Tefferi A, Lasho TL, Jimma T, Finke CM, Gangat N, Vaidya R; et al. (2012). "One thousand patients with primary myelofibrosis: the mayo clinic experience". Mayo Clin Proc. 87 (1): 25–33. doi:10.1016/j.mayocp.2011.11.001. PMC 3538387. PMID 22212965.
- ↑ Causes. The physician's guide to myelofibrosis 2016. http://nordphysicianguides.org/wp-content/uploads/2012/11/NORD_Physician_Guide_to_Myelofibrosis.pdf. Accessed on March 14, 2016
- ↑ Risk factors for myelofibrosis. Mayo clinic 2016. http://www.mayoclinic.org/diseases-conditions/myelofibrosis/basics/risk-factors/con-20027210. Accessed on March 7, 2016
- ↑ Diagnosis of myelofibrosis. Wikipedia 2016. https://en.wikipedia.org/wiki/Myelofibrosis. Accessed on March 8, 2016
- ↑ 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.
- ↑ 20.0 20.1 20.2 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
- ↑ World Health Organization (WHO) Diagnostic Criteria for Primary Myelofibrosis (PMF), Polycythemia Vera (PV), and Essential Thrombocythemia (ET). MPN Connect 2016. http://www.mpnconnect.com/pdf/who-diagnostic-criteria-myelofibrosis.pdf. Accessed on March 8, 2016
- ↑ Tefferi A, Thiele J, Orazi A, Kvasnicka HM, Barbui T, Hanson CA; et al. (2007). "Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel". Blood. 110 (4): 1092–7. doi:10.1182/blood-2007-04-083501. PMID 17488875.