National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Primary Myelofibrosis is not the name you expected.
Primary myelofibrosis (PMF) is a rare bone marrow disorder that is characterized by abnormalities in blood cell production (hematopoiesis) and scarring (formation of fibrous tissue) within the bone marrow. Bone marrow is the soft, spongy tissue that fills the center of most bones. Bone marrow contains specialized cells called hematopoietic stem cells that grow and eventually develop into one of the three main types of blood cells: red blood cells, white blood cells or platelets. In primary myelofibrosis, a change in the DNA of a single hematopoietic stem cell causes the abnormal cell to continually reproduce itself. Eventually, these abnormal cells crowd out normal, healthy cells in the marrow and, along with scarring within the marrow, disrupt the production of red and white blood cells and platelets.
The symptoms associated with primary myelofibrosis vary and are related to the abnormalities affecting blood cell production. Affected individuals may not have symptoms at the time of diagnosis (asymptomatic) may remain symptom-free for many years. Eventually, affected individuals may develop fatigue, fever, frequent infections, pale skin, night sweats and unexplained weight loss. An enlarged (spleen) is a common finding. An enlarged liver (hepatomegaly) may also occur.
In approximately 50 percent of patients, a mutation of the JAK2 gene has been detected. The exact role this abnormal gene plays in the development of the disorder is unknown.
Primary myelofibrosis belongs to a group of diseases known as the myeloproliferative neoplasms (MPNs). This group of disorders is characterized by the overproduction (proliferation) of one or more of the three main blood cell lines - red or white blood cells or platelets. Three other disorders are commonly classified as MPNs: chronic myeloid leukemia, essential thrombocythemia and polycythemia vera. Myelofibrosis may occur as a secondary characteristic of polycythemia vera or essential thrombocytyemia. Because the MPNs are characterized by uncontrolled cell growth, they may also be classified as blood cancers.
American Cancer Society, Inc.
- 250 Williams NW St
- Ste 6000
- Atlanta, GA 30303
- Tel: (404)320-3333
- Tel: (800)227-2345
- Website: http://www.cancer.org
Center for International Blood and Marrow Transplant Research
- Froedtert and the Medical College of Wisconsin Clinical Cancer Center
- 9200 W. Wisconsin Avenue
- Milwaukee, WI 53226
- Tel: (414)805-0700
- Fax: (414)805-0714
- Email: firstname.lastname@example.org
- Website: http://www.cibmtr.org/
Genetic and Rare Diseases (GARD) Information Center
- PO Box 8126
- Gaithersburg, MD 20898-8126
- Tel: (301)251-4925
- Fax: (301)251-4911
- Tel: (888)205-2311
- Website: http://rarediseases.info.nih.gov/GARD/
Leukemia & Lymphoma Society
- 3 International Drive
- Suite 200
- Rye Brook, NY 10573
- Tel: (914)949-5213
- Fax: (914)949-6691
- Tel: (800)955-4572
- Email: infocenter@LLS.org
- Website: http://www.LLS.org
MPN Education Foundation
- P O Box 4758
- Scottsdale, AZ 85261
- Tel: (480)443-1975
- Fax: (480)443-1154
- Email: email@example.com or firstname.lastname@example.org
- Website: http://www.mpninfo.org
MPN Research Foundation
- 180 N. Michigan Avenue, Suite 1870
- Chicago, IL 60601
- Tel: (312)683-7249
- Fax: (312)332-0840
- Email: RNunez@mpnresearchfoundation.org
- Website: http://www.mpnresearchfoundation.org
National Bone Marrow Transplant Link
- 20411 W. 12 Mile Rd
- Suite 108
- Southfield, MI 48076
- Tel: (248)358-1886
- Fax: (248)358-1889
- Tel: (800)546-5268
- Email: email@example.com
- Website: http://www.nbmtlink.org
National Cancer Institute
- 6116 Executive Blvd Suite 300
- Bethesda, MD 20892-8322
- Tel: (301)435-3848
- Tel: (800)422-6237
- Email: firstname.lastname@example.org
- Website: http://www.cancer.gov
Rare Cancer Alliance
- 1649 North Pacana Way
- Green Valley, AZ 85614
- Website: http://www.rare-cancer.org
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
Last Updated: 3/29/2016
Copyright 2016 National Organization for Rare Disorders, Inc.
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