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__NOTOC__
'''For patient information, click [[Myelofibrosis (patient information)|here]]'''
{{DiseaseDisorder infobox |
{{DiseaseDisorder infobox |
   Name          = Myelofibrosis |
   Name          = Myelofibrosis |
  ICD10          = {{ICD10|C|94|5|c|81}}, {{ICD10|D|47|1|d|37}} |
  ICD9          = {{ICD9|289.89}} |
   Image          = Myelofibrosis micro.jpg|
   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}}
 
{{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
 
==[[Myelofibrosis overview|Overview]]==
 
==[[Myelofibrosis historical perspective|Historical Perspective]]==


{{EH}}
==[[Myelofibrosis classification|Classification]]==


==Overview==
==[[Myelofibrosis pathophysiology|Pathophysiology]]==


'''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 causes|Causes]]==


==Presentation==
==[[Myelofibrosis differential diagnosis|Differentiating Myelofibrosis from other Diseases]]==
The [[bone marrow]] is replaced by collagen fibrosis, impairing the patient's ability to generate new blood cells resulting in a progressive anemia. 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]]. The mean survival is 5 years and causes of death include infection, bleeding, organ failure, portal hypertension, and leukemic transformation.


==Diagnosis==
==[[Myelofibrosis epidemiology and demographics|Epidemiology and Demographics]]==
Diagnosis is based upon:
# Normochromic normocytic anaemia
# Red cell poikilocytosis on blood film (tear drop RBCs)
# JAK 2 mutation on Val 617 Phe locus in 50%
# Raised lactate dehydrogenase
# Raised neutrophil alkaline phosphatase score
# Bone marrow biopsy may show increased cellularity and fibrosis


==Diagnostic Findings==
==[[Myelofibrosis risk factors|Risk Factors]]==


===MRI===
==[[Myelofibrosis screening|Screening]]==


([http://www.radswiki.net Images courtesy of RadsWiki])
==[[Myelofibrosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==


<gallery>
==Diagnosis==
Image:Myelofibrosis_MRI_001.jpg|Myelofibrosis
[[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]]
Image:Myelofibrosis_MRI_002.jpg|Myelofibrosis
</gallery>


<gallery>
==Treatment==
Image:Myelofibrosis_MRI_003.jpg|Myelofibrosis
[[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]]
Image:Myelofibrosis_MRI_004.jpg|Myelofibrosis
</gallery>


== References ==
==Case Studies==
{{reflist}}
[[Myelofibrosis case study one|Case # 1]]


<br>
{{Hematology}}
{{Hematological malignancy histology}}
{{SIB}}


==Related chapters==
* [[Myelophthisic anemia]]


[[Category:Medicine]]
[[Category:Hematology]]
[[Category:Hematology]]
[[Category:Oncology]]
[[Category:Oncology]]
 
[[Category:Neurology]]
[[bn:মজ্জাকাঠিন্য]]
[[Category:Neurosurgery]]
[[de:Osteomyelofibrose]]
[[Category:Up-To-Date]]
[[fr:Splénomégalie myéloïde]]
[[it:Mielofibrosi]]
[[pl:Mielofibroza]]
 
{{WH}}
{{WS}}

Latest revision as of 06:34, 28 July 2020

For patient information, click here

Template:DiseaseDisorder infobox

Myelofibrosis Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Myelofibrosis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

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Risk calculators and risk factors for Myelofibrosis

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

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Myelofibrosis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case # 1


Related chapters