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| | '''For patient information, click [[Myelofibrosis (patient information)|here]]''' |
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| {{DiseaseDisorder infobox | | | {{DiseaseDisorder infobox | |
| Name = Myelofibrosis | | | Name = Myelofibrosis | |
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| MeshID = D009191 | | | MeshID = D009191 | |
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| {{Search infobox}} | | {{Myelofibrosis}} |
| {{CMG}} | | {{CMG}} |
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| {{EH}}
| | ==[[Myelofibrosis overview|Overview]]== |
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| ==Overview== | | ==[[Myelofibrosis classification|Classification]]== |
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| '''Myelofibrosis with myeloid metaplasia''', also known as '''agnogenic myeloid metaplasia''', '''chronic idiopathic myelofibrosis''', and '''primary myelofibrosis''',<ref>Older terms include "myelofibrosis with myeloid metaplasia" and "agnogenic myeloid metaplasia". The [[World Health Organization]] utilizes the name "chronic idiopathic myelofibrosis", while the International Working Group on Myelofibrosis Research and Treatment calls the disease "primary myelofibrosis".</ref> was first described in 1879 and is currently classified as a [[myeloproliferative disease]] caused by the growth and proliferation of an abnormal [[bone marrow]] stem cell, resulting in the [[fibrosis|replacement of the bone marrow with fibrous connective tissue]]. An eponym for the disease is Heuck-Assmann disease, or Assmann's Disease.
| | ==[[Myelofibrosis historical perspective|Historical Perspective]]== |
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| ==Epidemiology== | | ==[[Myelofibrosis pathophysiology|Pathophysiology]]== |
| The disorder usually develops slowly, in people over 50 years old.
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| ==Pathophysiology== | | ==[[Myelofibrosis epidemiology and demographics|Epidemiology & Demographics]]== |
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| The bone marrow is replaced by collagen fibrosis, impairing the patient's ability to generate new blood cells resulting in a progressive [[pancytopenia]]. It is usually reactive following other [[myeloproliferative disorders]], such as [[polycythemia rubra vera]] or [[essential thrombocytosis]]. Extramedullary hematopoeisis occurs as the haemopoetic cells migrate away from the bone marrow, to the liver and spleen. Patients often have [[hepatosplenomegaly]] and [[poikilocytosis]].
| | ==[[Myelofibrosis epidemiology and demographics|Risk Factors]]== |
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| In primary myelofibrosis, a progressive scarring ([[fibrosis]]) of the bone marrow occurs. As a result, blood forms in sites other than the bone marrow, such as the [[liver]] and [[spleen]]. This causes an enlargement of these organs. The cause and risk factors are unknown. It commonly occurs in the spent phase of [[Polycythemia rubra vera]], possibly in response to the medication [[hydroxyurea]] poisoning the marrow.
| | ==[[Myelofibrosis screening|Screening]]== |
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| Genetic associations with [[JAK2]]<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> and [[Myeloproliferative leukemia virus oncogene|MPL]]<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> have been described.
| | ==[[Myelofibrosis causes|Causes]]== |
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| == Signs and symptoms == | | ==[[Myelofibrosis differential diagnosis|Differentiating Myelofibrosis from other Diseases]]== |
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| *Abdominal fullness related to an [[splenomegaly|enlarged spleen]] (splenomegaly).
| | ==[[Myelofibrosis natural history|Natural History, Complications & Prognosis]]== |
| *Bone pain
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| *Bruising and easy bleeding due to inadequate numbers of [[platelets]]
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| *Fatigue
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| *Increased susceptibility to infection, such as [[pneumonia]] or [[diarrhea]]
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| *Pallor and shortness of breath while doing physical work due to [[anemia]]
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| ==Diagnosis== | | ==Diagnosis== |
| | | [[Myelofibrosis history and symptoms|History & Symptoms]] | [[Myelofibrosis physical examination|Physical Examination]] | [[Myelofibrosis staging|Staging]] | [[Myelofibrosis laboratory tests|Lab Tests]] | [[Myelofibrosis electrocardiogram|Electrocardiogram]] | [[Myelofibrosis chest x ray|Chest X Ray]] | [[Myelofibrosis CT|CT]] | [[Myelofibrosis MRI|MRI]] | [[Myelofibrosis echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Myelofibrosis other imaging findings|Other Imaging Findings]] | [[Myelofibrosis other diagnostic studies|Other Diagnostic Studies]] |
| Diagnosis is based upon:
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| * [[Normochromic]] normocytic [[anaemia]]
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| * Red cell [[poikilocytosis]] on blood film (tear-drop shaped RBCs)
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| * JAK 2 mutation on Val 617 Phe locus in 50%
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| * Raised levels of [[lactate dehydrogenase]]
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| * Raised neutrophil alkaline phosphatase score
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| * [[Bone marrow biopsy]] may show increased cellularity and fibrosis
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| The patient with the myelofibrosis should be tested for [[Tuberculosis]] even if the patient doesn't manifest the symptoms of TB. There is strong evidence of myelofibrois improvement with the antituberculous treatment.
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| ==Diagnostic Findings==
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| ===MRI===
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| ([http://www.radswiki.net Images courtesy of RadsWiki])
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| <gallery>
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| Image:Myelofibrosis_MRI_001.jpg|Myelofibrosis
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| Image:Myelofibrosis_MRI_002.jpg|Myelofibrosis
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| </gallery>
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| <gallery>
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| Image:Myelofibrosis_MRI_003.jpg|Myelofibrosis
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| Image:Myelofibrosis_MRI_004.jpg|Myelofibrosis
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| </gallery>
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| ==Treatment== | | ==Treatment== |
| | [[Myelofibrosis medical therapy|Medical Therapy]] | [[Myelofibrosis surgery|Surgery]] | [[Myelofibrosis primary prevention|Primary Prevention]] | [[Myelofibrosis secondary prevention|Secondary Prevention]] |
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| The only known cure is allogeneic [[stem cell transplantation]], but this approach involves significant risks.<ref name="pmid15725078">{{cite journal |author=Cervantes F |title=Modern management of myelofibrosis |journal=Br. J. Haematol. |volume=128 |issue=5 |pages=583–92 |year=2005 |month=March |pmid=15725078 |doi=10.1111/j.1365-2141.2004.05301.x |url=http://dx.doi.org/10.1111/j.1365-2141.2004.05301.x}}</ref>
| | ==Related chapters== |
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| Other treatment options are largely supportive, and do not alter the course.<ref name="pmid18185525">{{cite journal |author=Kröger N, Mesa RA |title=Choosing between stem cell therapy and drugs in myelofibrosis |journal=Leukemia |volume=22 |issue=3 |pages=474–86 |year=2008 |month=March |pmid=18185525 |doi=10.1038/sj.leu.2405080 |url=http://dx.doi.org/10.1038/sj.leu.2405080}}</ref> These options may include regular [[folic acid]], [[allopurinol]] or [[blood transfusion]]s. [[Dexamethasone]], alpha-[[interferon]] and [[hydroxycarbamide]] may play a role.
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| [[Lenalidomide]] and [[Thalidomide]] may be used in its treatment, though they can cause [[gout]] and leave the patient susceptible to diseases such as [[pneumonia]].
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| Frequent [[blood transfusions]] may also be required.
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| ==Prognosis==
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| Myelofibrosis leads to progressive bone marrow failure. The mean survival is five years and causes of death include infection, bleeding, organ failure, portal hypertension, and leukemic transformation.
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| == References ==
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| {{reflist|2}}
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| ==See also==
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| * [[Myelophthisic anemia]] | | * [[Myelophthisic anemia]] |
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| {{Hematology}} | | {{Hematology}} |
| {{Hematological malignancy histology}} | | {{Hematological malignancy histology}} |
| {{SIB}}
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| | [[Category:Disease]] |
| | [[Category:Types of cancer]] |
| [[Category:Hematology]] | | [[Category:Hematology]] |
| [[Category:Oncology]] | | [[Category:Oncology]] |