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Primary MF, myeloid metaplasia
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- Normally the bone marrow is a nursery in which blood cells (white, red, and platelets) are born and mature. The mature cells are then released into the blood stream for a variety of purposes (i.e., fighting infections, carrying oxygen, fighting cancers, clotting of blood).
- In MF, which is often seen in those 50 and over, collagen-based fibrous tissue (scar) invades and occupies the space where blood cells are normally produced, eliminating the space reserved for normal growth and maturation. The red blood cells released are abnormally shaped (teardrop rather than round) and sluggish, while the white blood cells are numerous in number and too immature to function normally.
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- The patient may experience abdominal, bone or joint pain, weakness, weight loss, or pallor of skin (i.e., anemia develops due to abnormal red cells).
- The manufacturing of blood cells shifts to the spleen and the liver and, as a result, they enlarge abnormally.
- The bone density may also increase (osteosclerosis).
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- The most common form of MF is called idiopathic or primary MF, the cause for which is unknown, but certain growth stimulating factors (TGF-beta and PDGF) are being studied as possible contributors to the overgrowth of invasive fibrous tissue.
- It may also precede other disorders, such as Polycythemia Vera, thrombocytemia, Acute Leukemia, and chronic myeloid leukemia.
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- Diagnosis is made by a hematologist, and may include a blood analysis and a bone marrow biopsy.
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- Aims to increase the number of mature cells, and may include hormonal therapy (e.g., erythropoetin, androgens), transfusions of healthy cells, and chemotherapy. In some patients, removing the spleen (splenectomy) may be advised.
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