"Myelofibrosis" redirects here. It is not to be confused with fibromyalgia.
Medical condition
Primary myelofibrosis
Other names
PMF, Overt PMF, Myelofibrosis
Specialty
Oncology and Hematology
Primary myelofibrosis (PMF) is a rare bone marrow blood cancer.[1] It is classified by the World Health Organization (WHO) as a type of myeloproliferative neoplasm, a group of cancers in which there is activation and growth of mutated cells in the bone marrow. This is most often associated with a somatic mutation in the JAK2, CALR, or MPL genes. In PMF, the bony aspects of bone marrow are remodeled in a process called osteosclerosis; in addition, fibroblast secrete collagen and reticulin proteins that are collectively referred to as (fibrosis). These two pathological processes compromise the normal function of bone marrow resulting in decreased production of blood cells such as erythrocytes (red cells), granulocytes and megakaryocytes, the latter cells responsible for the production of platelets.
Signs and symptoms include fever, night sweats, bone pain, fatigue, and abdominal pain. Increased infections, bleeding and an enlarged spleen (splenomegaly) are also hallmarks of the disease. Patients with myelofibrosis have an increased risk of acute meyloid leukemia and frank bone marrow failure.
In 2016, prefibrotic primary myelofibrosis was formally classified as a distinct condition that progresses to overt PMF in many patients, the primary diagnostic difference being the grade of fibrosis.[2]
^"Myelofibrosis Facts" (PDF). The Leukemia and Lymphoma Society. Retrieved 5 October 2014.
^Cite error: The named reference treatmentalgo was invoked but never defined (see the help page).
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Primarymyelofibrosis (PMF) is a rare bone marrow blood cancer. It is classified by the World Health Organization (WHO) as a type of myeloproliferative...
Prefibrotic primarymyelofibrosis (Pre-PMF) is a rare blood cancer, classified by the World Health Organization as a distinct type of myeloproliferative...
CALR, TET2, and MPL gene markers. In rare cases, some MPNs such as primarymyelofibrosis may accelerate and turn into acute myeloid leukemia. MPNs are classified...
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criteria for polycythemia vera, essential thrombocythemia, and primarymyelofibrosis: recommendations from an ad hoc international expert panel". Blood...
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symptoms in adults with primarymyelofibrosis (also known as chronic idiopathic myelofibrosis), post-polycythaemia-vera myelofibrosis, or post-essential thrombocythaemia...
cells, such as polycythemia vera, essential thrombocytosis, and in primarymyelofibrosis. Lower than typical levels are found in pathologies that involve...
Presidential Management Fellows Program, a US government fellowship Primarymyelofibrosis, a disease affecting the bone marrow. Probability mass function...
August 2019. Myelofibrosis is a myeloid cancer associated with anemia, splenomegaly, and constitutional symptoms. Patients with myelofibrosis frequently...
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multiple other underlying neoplasms such as polycythemia vera (PV), myelofibrosis, thrombocythemia, or, in rare cases, solid tumors. Alternative root...
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2017). "Downregulation of GATA1 drives impaired hematopoiesis in primarymyelofibrosis". The Journal of Clinical Investigation. 127 (4): 1316–1320. doi:10...
JAK2-negative/MPL-negative patients with essential thrombocythemia and primarymyelofibrosis, which makes CALR mutations the second most common in myeloproliferative...
bone marrow osteoprogenitors increase in the advanced stages of primarymyelofibrosis". Haematologica. 94 (1): 127–30. doi:10.3324/haematol.13598. PMC 2625423...
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Hemopexin Orosomucoid Negative Serum albumin Transferrin Lymphatic system Primary lymphoid organs Thymus - Site of T cell maturation Bone marrow - Site of...
2017). "Downregulation of GATA1 drives impaired hematopoiesis in primarymyelofibrosis". The Journal of Clinical Investigation. 127 (4): 1316–1320. doi:10...
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