Primary Myelofibrosis

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

Myelofibrosis and Myeloid Metaplasia
Agnogenic Myeloid Metaplasia (AMM)
Primary Myelofibrosis (PMF)
Chronic Idiopathic Myelofibrosis (CIMF)
IM
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 Ave
3rd Floor
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
Internet: http://www.cancer.org

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
Email: marchettim@smatteo.pv.it
Internet: http://www.myelofibrosis.net

CMPD Education Foundation
PO Box 4758
Scottsdale, AZ 85261
Tel: (480)443-1975
Fax: (480)443-1154
Email: jniblack@mpdinfo.org
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: roberttollen@gmail.com or mensabrain@aol.com
Internet: http://www.MPDSUPPORT.ORG

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

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

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.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated: 4/14/2008
Copyright 1986, 1987, 1988, 1989, 1996, 2000, 2002, 2008 National Organization for Rare Disorders, Inc.

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