Myelofibrosis: Difference between revisions

	Myelofibrosis Microchapters
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Latest revision as of 06:34, 28 July 2020

For patient information, click here

Template:DiseaseDisorder infobox

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, M.D.[2]; Mohamad Alkateb, MBBCh [3]; Sujit Routray, M.D. [4]

Synonyms and keywords: Agnogenic myeloid metaplasia; AMM; Myeloid metaplasia; Myeloid metaplasia, NOS; Myelofibrosis with myeloid metaplasia; MMM; Assmann's disease; Chronic granulocytic-megakaryocytic myelosis; Chronic granulocytic megakaryocytic myelosis; Megakaryocytic myelosclerosis; Chronic idiopathic myelofibrosis; CIMF; Heuck-Assmann disease; Idiopathic myelofibrosis; Primary myelofibrosis; PMF; Prefibrotic primary myelofibrosis; Secondary myelofibrosis; Myelosclerosis; Osteomyelofibrosis; Osteomyelosclerosis; Myeloproliferative disorder

Diagnosis

Treatment

Case Studies

Case # 1

Related chapters

Myelophthisic anemia

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+__NOTOC__
+'''For patient information, click [[Myelofibrosis (patient information)|here]]'''
 {{DiseaseDisorder infobox |
    Name           = Myelofibrosis |
-  ICD10          = {{ICD10|C|94|5|c|81}}, {{ICD10|D|47|1|d|37}} |
-  ICD9           = {{ICD9|289.89}} |
    Image          = Myelofibrosis micro.jpg|
-   Caption        = Bone marrow: Myelofibrosis: High mag H&E excellent example of myelofibrosis. <br> <small> [http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small> |
+   Caption        = Bone marrow: Myelofibrosis: High mag HandE excellent example of myelofibrosis. <br> <small> [http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small> |
-  ICDO           = 9932, 9961 |
-  OMIM           = |
-  DiseasesDB     = 8616 |
-  MedlinePlus    = |
-  eMedicineSubj  = |
-  eMedicineTopic = |
-  MeshID          = D009191 |
 }}
-{{Search infobox}}
+{{Myelofibrosis}}
-{{CMG}}
+{{CMG}} {{AE}} {{Sab}}; {{MJK}}; {{SR}}
-{{EH}}
+{{SK}} Agnogenic myeloid metaplasia; AMM; Myeloid metaplasia; Myeloid metaplasia, NOS; Myelofibrosis with myeloid metaplasia; MMM; Assmann's disease; Chronic granulocytic-megakaryocytic myelosis; Chronic granulocytic megakaryocytic myelosis; Megakaryocytic myelosclerosis; Chronic idiopathic myelofibrosis; CIMF; Heuck-Assmann disease; Idiopathic myelofibrosis; Primary myelofibrosis; PMF; Prefibrotic primary myelofibrosis; Secondary myelofibrosis; Myelosclerosis; Osteomyelofibrosis; Osteomyelosclerosis; Myeloproliferative disorder
-==Overview==
+==[[Myelofibrosis overview|Overview]]==
-'''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]]==
-==Epidemiology==
+==[[Myelofibrosis classification|Classification]]==
-The disorder usually develops slowly, in people over 50 years old.
-==Pathophysiology==
+==[[Myelofibrosis pathophysiology|Pathophysiology]]==
-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 causes|Causes]]==
-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 differential diagnosis|Differentiating Myelofibrosis from other Diseases]]==
-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 epidemiology and demographics|Epidemiology and Demographics]]==
-== Signs and symptoms ==
+==[[Myelofibrosis risk factors|Risk Factors]]==
-*Abdominal fullness related to an [[splenomegaly|enlarged spleen]] (splenomegaly).
-*Bone pain
-*Bruising and easy bleeding due to inadequate numbers of [[platelets]]
-*Fatigue
-*Increased susceptibility to infection, such as [[pneumonia]] or [[diarrhea]]
-*Pallor and shortness of breath while doing physical work due to [[anemia]]
-==Diagnosis==
+==[[Myelofibrosis screening|Screening]]==
-Diagnosis is based upon:
+==[[Myelofibrosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
-* [[Normochromic]] normocytic [[anaemia]]
+==Diagnosis==
-* Red cell [[poikilocytosis]] on blood film (tear-drop shaped RBCs)
+[[Myelofibrosis diagnostic study of choice|Diagnostic Study of Choice]] | [[Myelofibrosis history and symptoms|History and Symptoms]] | [[Myelofibrosis physical examination|Physical Examination]] | [[Myelofibrosis laboratory tests|Laboratory Findings]] | [[Myelofibrosis electrocardiogram|Electrocardiogram]] | [[Myelofibrosis x ray|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]]
-* JAK 2 mutation on Val 617 Phe locus in 50%
-* Raised levels of [[lactate dehydrogenase]]
-* Raised neutrophil alkaline phosphatase score
-* [[Bone marrow biopsy]] may show increased cellularity and fibrosis
-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.
-==Diagnostic Findings==
-===MRI===
-([http://www.radswiki.net Images courtesy of RadsWiki])
-<gallery>
-Image:Myelofibrosis_MRI_001.jpg|Myelofibrosis
-Image:Myelofibrosis_MRI_002.jpg|Myelofibrosis
-</gallery>
-<gallery>
-Image:Myelofibrosis_MRI_003.jpg|Myelofibrosis
-Image:Myelofibrosis_MRI_004.jpg|Myelofibrosis
-</gallery>
 ==Treatment==
+[[Myelofibrosis medical therapy|Medical Therapy]] | [[Myelofibrosis surgery|Surgery]] | [[Myelofibrosis primary prevention|Primary Prevention]] | [[Myelofibrosis secondary prevention|Secondary Prevention]] | [[Myelofibrosis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Myelofibrosis future or investigational therapies|Future or Investigational Therapies]]
-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>
+==Case Studies==
+[[Myelofibrosis case study one|Case # 1]]
-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.
-[[Lenalidomide]] and [[Thalidomide]] may be used in its treatment, though they can cause [[gout]] and leave the patient susceptible to diseases such as [[pneumonia]].
-Frequent [[blood transfusions]] may also be required.
-==Prognosis==
-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.
-== References ==
-{{reflist}}
-<br>
-{{Hematology}}
-{{Hematological malignancy histology}}
-{{SIB}}
+==Related chapters==
+* [[Myelophthisic anemia]]
+[[Category:Medicine]]
 [[Category:Hematology]]
 [[Category:Oncology]]
+[[Category:Neurology]]
-[[bn:মজ্জাকাঠিন্য]]
+[[Category:Neurosurgery]]
-[[de:Osteomyelofibrose]]
+[[Category:Up-To-Date]]
-[[fr:Splénomégalie myéloïde]]
-[[it:Mielofibrosi]]
-[[pl:Mielofibroza]]
-{{WH}}
-{{WS}}