The treatment goal for most patients with MF is to relieve symptoms, reduce an enlarged spleen, improve blood cell counts i. What You Should Do Talk with your doctor about your diagnostic tests and what the results mean. Talk with your doctor about all your treatment options, side effects, and the results you can expect from treatment. Ask your doctor whether a clinical trial is a good treatment option for you.
How Does MF Develop? Red blood cells carry oxygen to the tissues. White blood cells fight infection. Mayo Clinic; Myeloproliferative neoplasms. National Comprehensive Cancer Network. Accessed Dec. Hoffman R, et al. In: Hematology: Basic Principles and Practice. Elsevier; Tefferi A. Primary myelofibrosis: update on diagnosis, risk-stratification and management. American Journal of Hematology. Palliative care. Warner KJ. Allscripts EPSi. Mayo Clinic. Gangat N, et al.
Myelofibrosis biology and contemporary management. British Journal of Hematology. This results in anaemia, low platelet counts and the production of blood cells in areas outside the bone marrow for example in the spleen and liver, which become enlarged as a result. Primary myelofibrosis is a rare chronic disorder diagnosed in an estimated 1 per , population. It can occur at any age but is usually diagnosed later in life, between the ages of 60 and 70 years.
The cause of primary myelofibrosis remains largely unknown. Long-term exposure to high levels of benzene or very high doses of ionising radiation may increase the risk of primary myelofibrosis in a small number of cases. Around one third of people with myelofibrosis have been previously diagnosed with polycythaemia post-polycythaemic myelofibrosis or essential thrombocythaemia post-ET myelofibrosis. Around 20 per cent of people have no symptoms of primary myelofibrosis when they are first diagnosed and the disorder is picked up incidentally as a result of a routine blood test.
For others, symptoms develop gradually over time. Symptoms of anaemia are common and include unexplained tiredness, weakness, shortness of breath and palpitations. Other nonspecific symptoms include fever, unintended weight loss, pruritus generalised itching and excess sweating, especially at night.
Virtually all patients with primary myelofibrosis have an enlarged spleen splenomegaly when they are first diagnosed. In around a third of cases the spleen is very enlarged. Common symptoms include feelings of discomfort, pain or fullness in the upper left-side of the abdomen.
An enlarged spleen may also cause pressure on your stomach causing a feeling of fullness, indigestion and a loss of appetite. Abdominal discomfort can also result from an enlarged liver hepatomegaly , which occurs in around two-thirds of cases. Primary myelofibrosis is diagnosed using a combination of a physical examination showing the presence of an enlarged spleen, blood tests and a bone marrow examination. Primary myelofibrosis is only diagnosed when other causes of marrow fibrosis including leukaemia, lymphoma, other types of cancer that have spread to the bone marrow have been ruled out.
People with primary myelofibrosis commonly present with varying degrees of anaemia. Higher than normal numbers of white cells and platelets may be found in the early stages of this disorder, but low white cell and platelet counts are common in more advanced disease.
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