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Myelofibrosis (MF) - also sometimes known as agnogenic myeloid metaplasia - affects a person's bone marrow, the factory in our body that produces blood cells. When someone has MF, his or her bone marrow becomes scarred and fibrosed. This happens because the bone marrow contains too many fibroblasts, which are the cells that produce connective tissue, scar and fibrous tissue. The excess fibroblasts scar the bone marrow and stop the bone marrow from making blood cells. Myelofibrosis usually occurs in people over sixty years old. It can either be primary (or idiopathic), meaning that the cause is unknown. MF can also be secondary. In this case fibrosis develops as a result of another condition, often after Essential Thrombocythaemia (ET) or Polycythaemia Vera (PV). Secondary MF sometimes occurs as a reaction to other disorders, as a result of autoimmune or inflammatory conditions, cancers such as Hodgkin’s Disease or tuberculosis. Recent research shows that about 50% of patients have a mutation in a protein called JAK2 - a protein that regulates blood cell production. This mutation may result from viral infections or radiation damage, but more research needs to be done to determine the causes of MF.
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