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==Overview== | ==Overview== | ||
Myelofibrosis is a hematological disorder where there is replacement of bone marrow with [[collagen|collagenous connective tissue]] and progressive [[fibrosis]], or the replacement of the marrow with scar tissue. It is also classified as a myeloproliferative disorder.<ref name=overviewofmyelofibrosis1>Myelofibrosis. Dr Henry Knipe ◉ and Dr Yuranga Weerakkody et al. Radiopaedia 2016. http://radiopaedia.org/articles/myelofibrosis. Accessed on March 7, 2016</ref><ref name=myelofibrosisoverviewwiki1>Myelofibrosis. Wikipedia 2016. https://en.wikipedia.org/wiki/Myelofibrosis. Accessed on March 7, 2016</ref> 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.<ref name=myelofibrosisoverviewwiki1>Myelofibrosis. Wikipedia 2016. https://en.wikipedia.org/wiki/Myelofibrosis. Accessed on March 7, 2016</ref> | Myelofibrosis is a hematological disorder where there is replacement of bone marrow with [[collagen|collagenous connective tissue]] and progressive [[fibrosis]], or the replacement of the marrow with scar tissue. It is also classified as a myeloproliferative disorder.<ref name=overviewofmyelofibrosis1>Myelofibrosis. Dr Henry Knipe ◉ and Dr Yuranga Weerakkody et al. Radiopaedia 2016. http://radiopaedia.org/articles/myelofibrosis. Accessed on March 7, 2016</ref><ref name=myelofibrosisoverviewwiki1>Myelofibrosis. Wikipedia 2016. https://en.wikipedia.org/wiki/Myelofibrosis. Accessed on March 7, 2016</ref> 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.<ref name=myelofibrosisoverviewwiki1>Myelofibrosis. Wikipedia 2016. https://en.wikipedia.org/wiki/Myelofibrosis. Accessed on March 7, 2016</ref> | ||
==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'.<ref name="Tefferi2007">{{cite journal|last1=Tefferi|first1=A|title=The history of myeloproliferative disorders: before and after Dameshek|journal=Leukemia|volume=22|issue=1|year=2007|pages=3–13|issn=0887-6924|doi=10.1038/sj.leu.2404946}}</ref> | |||
==Classification== | |||
Based on the origin, myelofibrosis may be classified into two subtypes: '''primary''' and '''secondary'''.<ref name=classmyelof1>Classification of myelofibrosis. Dr Henry Knipe and Dr Yuranga Weerakkody et al. Radiopaedia 2016. http://radiopaedia.org/articles/myelofibrosis. Accessed on March 14, 2016</ref> | |||
==Pathophysiology== | |||
Myelofibrosis is a [[Clone (cell biology)|clonal]] neoplastic disorder of [[hematopoiesis]], the formation of blood cellular components.<ref name=causesofmyelofibrosis1wiki1>Causes of myelofibrosis. Wikipedia 2016. https://en.wikipedia.org/wiki/Myelofibrosis. Accessed on March 7, 2016</ref> 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]]'', ''[[Calreticulin|CALR]]'', and ''[[Myeloproliferative leukemia virus oncogene|MPL]]''.<ref name="TefferiLasho2014">{{cite journal|last1=Tefferi|first1=A|last2=Lasho|first2=T L|last3=Finke|first3=C M|last4=Knudson|first4=R A|last5=Ketterling|first5=R|last6=Hanson|first6=C H|last7=Maffioli|first7=M|last8=Caramazza|first8=D|last9=Passamonti|first9=F|last10=Pardanani|first10=A|title=CALR vs JAK2 vs MPL-mutated or triple-negative myelofibrosis: clinical, cytogenetic and molecular comparisons|journal=Leukemia|volume=28|issue=7|year=2014|pages=1472–1477|issn=0887-6924|doi=10.1038/leu.2014.3}}</ref> | |||
==Causes== | |||
Common causes of myelofibrosis include [[mutation|genetic mutations]]. The genes involved are listed [[myelofibrosis pathophysiology|'''here''']].<ref name="TefferiLasho2014">{{cite journal|last1=Tefferi|first1=A|last2=Lasho|first2=T L|last3=Finke|first3=C M|last4=Knudson|first4=R A|last5=Ketterling|first5=R|last6=Hanson|first6=C H|last7=Maffioli|first7=M|last8=Caramazza|first8=D|last9=Passamonti|first9=F|last10=Pardanani|first10=A|title=CALR vs JAK2 vs MPL-mutated or triple-negative myelofibrosis: clinical, cytogenetic and molecular comparisons|journal=Leukemia|volume=28|issue=7|year=2014|pages=1472–1477|issn=0887-6924|doi=10.1038/leu.2014.3}}</ref><ref name="pmid15781101">{{cite journal |author=Baxter EJ, Scott LM, Campbell PJ, ''et al'' |title=Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders |journal=Lancet |volume=365 |issue=9464 |pages=1054–61 |year=2005 |pmid=15781101 |doi=10.1016/S0140-6736(05)71142-9 |url=http://linkinghub.elsevier.com/retrieve/pii/S0140-6736(05)71142-9}}</ref><ref name="pmid16834459">{{cite journal |author=Pikman Y, Lee BH, Mercher T, ''et al'' |title=MPLW515L is a novel somatic activating mutation in myelofibrosis with myeloid metaplasia |journal=PLoS Med. |volume=3 |issue=7 |pages=e270 |year=2006 |month=July |pmid=16834459 |pmc=1502153 |doi=10.1371/journal.pmed.0030270 |url=http://medicine.plosjournals.org/perlserv/?request=get-document&doi=10.1371/journal.pmed.0030270}}</ref> | |||
==Differentiating Myelofibrosis from other Diseases== | |||
Myelofibrosis must be differentiated from other diseases that cause diffuse bone sclerosis, such as [[sickle cell disease]], [[hyperthyroidism]], [[dysplasia|sclerosing bone dysplasia]], [[bone metastasis|osteoblastic metastases]], and [[Paget's disease]].<ref name=myelofibrosisdiffdiagnsosiseradio1>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</ref><ref name=Diffusebonysclerosisdifferentialdiagnosis1>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</ref> Myelofibrosis must be differentiated from other diseases that cause [[splenomegaly]], such as [[anemia]], [[CML]], [[polycythemia rubra vera]], [[cirrhosis]], [[infections]], [[neoplastic]], and [[lipid storage disorder|lipid storage disorders]].<ref name=myelofibrosisdiffdiagnsosiseradio1>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</ref><ref name=splenomegalydiffdiagnosisradio1>Splenomegaly. Dr Henry Knipe and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/''Italic text''articles/splenomegaly. Accessed on March 11, 2016</ref> | |||
==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.<ref name=epidemiologyofmyelofibrosis1radio1>Epidemiology of myelofibrosis. Dr Henry Knipe and Dr Yuranga Weerakkody et al. Radiopaedia 2016. http://radiopaedia.org/articles/myelofibrosis. Accessed on March 8, 2016</ref> Males are more commonly affected with myelofibrosis than females. The male to female ratio is approximately 1.5 to 1.<ref name="pmid22212965">{{cite journal| author=Tefferi A, Lasho TL, Jimma T, Finke CM, Gangat N, Vaidya R et al.| title=One thousand patients with primary myelofibrosis: the mayo clinic experience. | journal=Mayo Clin Proc | year= 2012 | volume= 87 | issue= 1 | pages= 25-33 | pmid=22212965 | doi=10.1016/j.mayocp.2011.11.001 | pmc=PMC3538387 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22212965 }} </ref> Myelofibrosis usually affects individuals of the Ashkenazi Jews race. African American, Latin American, and Asian individuals are less likely to develop myelofibrosis.<ref name=racemf1>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</ref> | |||
==Risk Factors== | |||
Common risk factors in the development of myelofibrosis may be age, [[myeloproliferative disorder|other myeloproliferative disorders]], [[radiation]], or industrial chemical exposure.<ref name=riskfactorsofmyelofibrosismayoclinic1>Risk factors for myelofibrosis. Mayo clinic 2016. http://www.mayoclinic.org/diseases-conditions/myelofibrosis/basics/risk-factors/con-20027210. Accessed on March 7, 2016</ref> | |||
==Natural History, Complications and Prognosis== | |||
Myelofibrosis has a very indolent course.<ref name=wikidiagn1osismf1>Diagnosis of myelofibrosis. Wikipedia 2016. https://en.wikipedia.org/wiki/Myelofibrosis. Accessed on March 8, 2016</ref> If left untreated, myelofibrosis may progress to develop [[acute myelogenous leukemia]], [[Blood clots|thrombohemorrhagic events]], and [[Bone marrow failure|progressive marrow failure]]. Common complications of myelofibrosis include [[infections]], [[bleeding]], [[hepatic failure]], [[heart failure]], and [[gout]].<ref name=complicatnmf1>Complications of myelofibrosis. US National Library of Medicine 2016. https://www.nlm.nih.gov/medlineplus/ency/article/000531.htm. Accessed on March 7, 2016</ref><ref name=signsandsymptomsofmyelofibrosiswiki1>Signs and symptoms of myelofibrosis. Wikipedia 2016. https://en.wikipedia.org/wiki/Myelofibrosis. Accessed on March 7, 2016</ref><ref name="KelleYıldız2015">{{cite journal|last1=Kelle|first1=Bayram|last2=Yıldız|first2=Fatih|last3=Paydas|first3=Semra|last4=Bagır|first4=Emine Kılıc|last5=Ergin|first5=Melek|last6=Kozanoglu|first6=Erkan|title=Coexistence of hypertrophic osteoarthropathy and myelofibrosis|journal=Revista Brasileira de Reumatologia (English Edition)|year=2015|issn=22555021|doi=10.1016/j.rbre.2014.11.004}}</ref><ref name=diseaseoverviewmf1>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</ref><ref name=complmf1radio1>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</ref> 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.<ref name=diseaseoverviewmf1>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</ref> | |||
==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.<ref name=diagnosticcriteriamyelofibrosis1>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</ref><ref name="pmid17488875">{{cite journal| author=Tefferi A, Thiele J, Orazi A, Kvasnicka HM, Barbui T, Hanson CA et al.| title=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. | journal=Blood | year= 2007 | volume= 110 | issue= 4 | pages= 1092-7 | pmid=17488875 | doi=10.1182/blood-2007-04-083501 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17488875 }} </ref> | |||
===Staging=== | |||
There is no established system for the staging of myelofibrosis.<ref name=diseaseoverviewmf1>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</ref> | |||
==References== | ==References== | ||
Revision as of 14:13, 15 March 2016
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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.