Primary Myelofibrosis

National Organization for Rare Disorders, Inc.

Important
It is possible that the main title of the report Primary Myelofibrosis is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • IM
  • PMF
  • agnogenic myeloid metaplasia (AMM)
  • chronic idiopathic myelofibrosis (CIMF)
  • myelofibrosis with myeloid metaplasia
  • idiopathic myelofibrosis

Disorder Subdivisions

  • None

General Discussion

Primary myelofibrosis 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 idiopathic 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 idiopathic 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.

Idiopathic myelofibrosis belongs to a group of diseases known as the myeloproliferative disorders (MPDs). 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 MPDs: chronic myeloid leukemia, essential thrombocythemia and polycythemia vera. Myelofibrosis may occur as a secondary characteristic of polycythemia vera or essential thrombocytyemia. Because the MPDs are characterized by uncontrolled cell growth, they may also be classified as blood cancers.

When the cause of myelofibrosis is unknown it is referred to as idiopathic or primary. In approximately 50 percent of cases, a mutation of the JAK2 gene has been detected. The exact role this abnormal gene plays in the development of the disorder is unknown.

Resources

Leukemia & Lymphoma Society
1311 Mamaroneck Avenue
Suite 310
White Plains, NY 10605
Tel: (914)949-5213
Fax: (914)949-6691
Tel: (800)955-4572
Email: infocenter@LLS.org
Internet: http://www.LLS.org

American Cancer Society, Inc.
1599 Clifton Road NE
Atlanta, GA 30329
USA
Tel: (404)320-3333
Tel: (800)227-2345
TDD: (866)228-4327
Internet: http://www.cancer.org

National Cancer Institute
6116 Executive Blvd, MSC 8322, Room 3036A
Bethesda, MD 20892-8322
USA
Tel: (301)435-3848
Tel: (800)422-6237
TDD: (800)332-8615
Internet: http://www.cancer.gov

Center for International Blood and Marrow Transplant Research
Medical College of Wisconsin
8701 Watertown Plank Road
Milwaukee, WI 53226
Tel: (414)456-8325
Fax: (414)456-6530
Email: mnugent@hpi.mcw.edu
Internet: http://www.ibmtr.org; http://www.cibmtr.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: info@nbmtlink.org
Internet: http://www.nbmtlink.org

Myeloproliferative Mailing List (MPD-SUPPORT-L)
3706 North Roosevelt Blvd.
Key West, FL 44030
USA
Tel: (305)295-4444
Fax: (305)295-3335
Email: lov2laf@bellsouth.net
Internet: http://www.mpdsupport.org

Italian Registry of Myelofibrosis with Myeloid Metaplasia
Laboratoria Di Informatica Medica
IRCCS Policlinico San Matteo
Viale Golgi 19
Pavia, Intl 27100
Italy
Tel: 800 279 656
Fax: 0382 503393
Tel: (800)
Email: marchettim@smatteo.pv.it
Internet: http://www.myelofibrosis.net

Rare Cancer Alliance
1649 North Pacana Way
Green Valley, AZ 85614
USA
Tel: (520)625-5495
Fax: (615)526-4921
Email: sharon.lane@rare-cancer.org
Internet: http://www.rare-cancer.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
TDD: (888)205-3223
Email: ordr@od.nih.gov
Internet: http://rarediseases.info.nih.gov/Default.aspx

CMPD Education Foundation
233 S. Wacker Drive, Suite 375
Chicago, IL 60606
Tel: (312)683-7243
Fax: (312)332-0840
Email: ian.sweet@homemail.com.au
Internet: http://www.mpdinfo.org

Myeloproliferative Disease Support and Free Daily Email Digest
2011 Flagler Ave.
Key West, FL 33040
USA
Tel: (305)295-4444
Email: listserv@listserv.aol.com
Internet: http://www.mpdsupport.org/

Myeloproliferative Disorder (MPD) Foundation
233 S. Wacker Drive
Suite 375
Chicago, IL 60606
Tel: (312)683-7243
Fax: (312)332-0840
Email: abrazeau@mpdfoundation.org
Internet: http://www.mpdfoundation.org

For a Complete Report

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html

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