Myelofibrosis
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| Myelofibrosis Classification and external resources | |
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| Bone marrow: Myelofibrosis: High mag H&E excellent example of myelofibrosis. Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology | |
| ICD-10 | C94.5, D47.1 |
| ICD-9 | 289.89 |
| ICD-O: | 9932, 9961 |
| DiseasesDB | 8616 |
| MeSH | D009191 |
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WikiDoc Resources for Myelofibrosis | |
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Most recent articles on Myelofibrosis Most cited articles on Myelofibrosis | |
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Powerpoint slides on Myelofibrosis | |
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Evidence Based Medicine | |
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Clinical Trials | |
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Ongoing Trials on Myelofibrosis at Clinical Trials.gov Trial results on Myelofibrosis Clinical Trials on Myelofibrosis at Google
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US National Guidelines Clearinghouse on Myelofibrosis NICE Guidance on Myelofibrosis
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Definitions | |
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Patient Resources / Community | |
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Patient resources on Myelofibrosis Discussion groups on Myelofibrosis Patient Handouts on Myelofibrosis Directions to Hospitals Treating Myelofibrosis Risk calculators and risk factors for Myelofibrosis
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Healthcare Provider Resources | |
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Causes & Risk Factors for Myelofibrosis | |
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Continuing Medical Education (CME) | |
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Experimental / Informatics | |
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Overview
Myelofibrosis with myeloid metaplasia, also known as agnogenic myeloid metaplasia, chronic idiopathic myelofibrosis, and primary myelofibrosis,[1] 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 replacement of the bone marrow with fibrous connective tissue. An eponym for the disease is Heuck-Assmann disease, or Assmann's Disease.
Epidemiology
The disorder usually develops slowly, in people over 50 years old.
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.
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.
Genetic associations with JAK2[2] and MPL[3] have been described.
Signs and symptoms
- Abdominal fullness related to an 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
Diagnosis is based upon:
- Normochromic normocytic anaemia
- Red cell poikilocytosis on blood film (tear-drop shaped RBCs)
- 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
Myelofibrosis MRI 001.jpg
Myelofibrosis |
Myelofibrosis MRI 002.jpg
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Myelofibrosis MRI 003.jpg
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Myelofibrosis MRI 004.jpg
Myelofibrosis |
Treatment
The only known cure is allogeneic stem cell transplantation, but this approach involves significant risks.[4]
Other treatment options are largely supportive, and do not alter the course.[5] These options may include regular folic acid, allopurinol or blood transfusions. 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
- ↑ 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".
- ↑ 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 (July 2006). "MPLW515L is a novel somatic activating mutation in myelofibrosis with myeloid metaplasia". PLoS Med. 3 (7): e270. doi:10.1371/journal.pmed.0030270. PMID 16834459.
- ↑ Cervantes F (March 2005). "Modern management of myelofibrosis". Br. J. Haematol. 128 (5): 583–92. doi:10.1111/j.1365-2141.2004.05301.x. PMID 15725078.
- ↑ Kröger N, Mesa RA (March 2008). "Choosing between stem cell therapy and drugs in myelofibrosis". Leukemia 22 (3): 474–86. doi:10.1038/sj.leu.2405080. PMID 18185525.
See also
Myeloid Hematological malignancy/leukemia histology (ICD-O 9590-9989, C81-C96, 200-208) | |||||||||||||||
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| CFU-GM/ and other granulocytes |
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| MEP |
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| CFU-Mast | Mastocytoma (Mast cell leukemia, Mast cell sarcoma, Systemic mastocytosis) | ||||||||||||||
| Multiple/unknown | AML (Acute panmyelosis with myelofibrosis, Myeloid sarcoma) · MP (Myelofibrosis) · Acute biphenotypic leukaemia | ||||||||||||||
| See also hematology, lymphoid malignancy | |||||||||||||||
WikiDoc Research Resources for Myelofibrosis | |
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| Articles on Myelofibrosis | Most recent articles on Myelofibrosis • Most cited articles on Myelofibrosis • Review articles on Myelofibrosis • Articles on Myelofibrosis in N Eng J Med, Lancet, BMJ |
| Media (Slides, Video, Images, MP3) on Myelofibrosis | Powerpoint slides on Myelofibrosis • Images of Myelofibrosis • Photos of Myelofibrosis • Podcasts & MP3s on Myelofibrosis • Videos on Myelofibrosis |
| Evidence Based Medicine Regarding Myelofibrosis | Cochrane Collaboration on Myelofibrosis • Bandolier on Myelofibrosis • TRIP on Myelofibrosis |
| Cost Effectiveness of Myelofibrosis | Cost Effectiveness of Myelofibrosis |
| Clinical Trials Involving Myelofibrosis | Ongoing Trials on Myelofibrosis at Clinical Trials.gov • Trial results on Myelofibrosis • Clinical Trials on Myelofibrosis at Google |
| Guidelines / Policies / Government Resources (FDA/CDC) Regarding Myelofibrosis | US National Guidelines Clearinghouse on Myelofibrosis • NICE Guidance on Myelofibrosis • NHS PRODIGY Guidance • FDA on Myelofibrosis • CDC on Myelofibrosis |
| Textbook Information on Myelofibrosis | Books and Textbook Information on Myelofibrosis |
| Pharmacology Resources on Myelofibrosis | Dosing of Myelofibrosis • Drug interactions with Myelofibrosis • Side effects of Myelofibrosis • Allergic reactions to Myelofibrosis • Overdose information on Myelofibrosis • Carcinogenicity information on Myelofibrosis • Myelofibrosis in pregnancy • Pharmacokinetics of Myelofibrosis • |
| Genetics, Pharmacogenomics, and Proteinomics of Myelofibrosis | Genetics of Myelofibrosis • Pharmacogenomics of Myelofibrosis • Proteomics of Myelofibrosis |
| Newstories on Myelofibrosis | Myelofibrosis in the news • Be alerted to news on Myelofibrosis • News trends on Myelofibrosis |
| Commentary on Myelofibrosis | Blogs on Myelofibrosis |
| Patient Resources on Myelofibrosis | Patient resources on Myelofibrosis • Discussion groups on Myelofibrosis • Patient Handouts on Myelofibrosis • Directions to Hospitals Treating Myelofibrosis • Risk calculators and risk factors for Myelofibrosis |
| Healthcare Provider Resources on Myelofibrosis | Symptoms of Myelofibrosis • Causes & Risk Factors for Myelofibrosis • Diagnostic studies for Myelofibrosis • Treatment of Myelofibrosis |
| Continuing Medical Education (CME) Programs on Myelofibrosis | CME Programs on Myelofibrosis |
| International Resources on Myelofibrosis | Myelofibrosis en Espanol • Myelofibrosis en Francais |
| Business Resources on Myelofibrosis | Myelofibrosis in the Marketplace • Patents on Myelofibrosis |
| Informatics Resources on Myelofibrosis | List of terms related to Myelofibrosis |
de:Osteomyelofibroseit:Mielofibrosi
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
