Primary myelofibrosis
myeloid neoplasm that is located in the bone marrow which results in bone marrow being replaced by fibrous (scar) tissue
Primary myelofibrosis (PMF) is a rare bone marrow blood cancer. 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, fibroblasts 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 are 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.
This brief starts where responsible research should: with the source description of “Primary myelofibrosis” as myeloid neoplasm that is located in the bone marrow which results in bone marrow being replaced by fibrous (scar) tissue. Everything that follows is an evidence route, not borrowed authority.
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Named sources, stable identifiers and responsible institutions provide the strongest route from overview to verifiable evidence. The source revision retrieved here is dated Aug 12, 2026. The linked authority identifier is Q1752571. None of the 0 selected statements returned an explicit reference. The first chronological checks are 2016.
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This entry incorporates text from “Primary myelofibrosis” on English Wikipedia. Contributors are listed in the page history. Text is available under the Creative Commons Attribution-ShareAlike 4.0 License. Selected authority identifiers and statements are retrieved from Wikidata under CC0; their references and qualifiers remain part of the verification path.