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.
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.
Leukemia & Lymphoma Society
1311 Mamaroneck Avenue
New York, NY 10605
American Cancer Society, Inc.
1599 Clifton Road NE
Atlanta, GA 30329
National Cancer Institute
6116 Executive Blvd
Bethesda, MD 20892-8322
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
National Bone Marrow Transplant Link
20411 W. 12 Mile Rd
Southfield, MI 48076
Italian Registry of Myelofibrosis with Myeloid Metaplasia
Laboratoria Di Informatica Medica
IRCCS Policlinico San Matteo
Viale Golgi 19
Pavia, Intl 27100
Tel: 800 279 656
Fax: 0382 503393
Rare Cancer Alliance
1649 North Pacana Way
Green Valley, AZ 85614
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
CMPD Education Foundation
P.O. Box 4758
Scottsdale, AZ 85261
Myeloproliferative Disease Support and Daily Email Digest
2011 Flagler Ave.
Key West, FL 33040
MPN Research Foundation
180 N. Michigan Avenue, Suite 1870
Chicago, IL 60606
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Last Updated: 4/14/2008
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