Primary myelofibrosis
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Primary myelofibrosis is a condition characterised by the buildup of scar tissue (fibrosis) in the bone marrow, the tissue that produces blood cells. Because of the fibrosis, the bone marrow is unable to make enough normal blood cells. The shortage of blood cells causes many of the signs and symptoms of primary myelofibrosis.
Descrizione
Primary myelofibrosis is a condition characterised by the buildup of scar tissue (fibrosis) in the bone marrow, the tissue that produces blood cells. Because of the fibrosis, the bone marrow is unable to make enough normal blood cells. The shortage of blood cells causes many of the signs and symptoms of primary myelofibrosis.
Initially, most people with primary myelofibrosis have no signs or symptoms. Eventually, fibrosis can lead to a reduction in the number of red blood cells, white blood cells, and platelets. A shortage of red blood cells (anaemia) often causes extreme tiredness (fatigue) or shortness of breath. A loss of white blood cells can lead to an increased number of infections, and a reduction of platelets can cause easy bleeding or bruising.
Because blood cell formation (hematopoiesis) in the bone marrow is disrupted, other organs such as the spleen or liver may begin to produce blood cells. This process, called extramedullary hematopoiesis, often leads to an enlarged spleen (splenomegaly) or an enlarged liver (hepatomegaly). People with splenomegaly may feel pain or fullness in the abdomen, especially below the ribs on the left side. Other common signs and symptoms of primary myelofibrosis include fever, night sweats, and bone pain.
Primary myelofibrosis is most commonly diagnosed in people aged 50 to 80 but can occur at any age.
Modelli di ereditarietà
The source lists the following possible inheritance patterns: Not inherited. More than one pattern can apply in different cases.
Related genes
Altri nomi
Agnogenic myeloid metaplasia
Chronic idiopathic myelofibrosis
Idiopathic myelofibrosis
Myelofibrosis with myeloid metaplasia
Myeloid metaplasia
Related patient information and medicines
Patient information leaflet
Mielofibrosi
Myelofibrosis is a condition affecting the bone marrow and its blood cell production. What is mye... Myelofibrosis belongs to a group of diseases called myeloproliferative disorders. Myelofibrosis is a serious bone marrow disorder that disrupts your body's normal production of blood cells. What causes myelofibrosis. Myelofibrosis which occurs.
Patient information leaflet
Milia
Milia are very small, raised, pearly-white or yellowish bumps on the skin. They are most often seen on the skin around the cheeks, nose, eyes and eyelids, forehead and chest. They usually disappear by 12 weeks of age. Milia primarie These are milia that can occur in both children and adults. They occur in an area of skin that has otherwise been.
Patient information leaflet
Leucemia mieloide cronica
Leucemia mieloide cronica (LMC) is sometimes called chronic myelogenous leukaemia, chronic granulocytic leucemia, or chronic myelocytic leukaemia. What is chronic myeloid leukaemia. In this phase there are more than 30% blast cells in the blood and bone marrow. Very rarely, CML develops into a condition called myelofibrosis. This means that the.
Patient information leaflet
Policitemia rubra vera
Polycythaemia rubra vera (PRV) is a myeloproliferative disorder, which means the bone marrow makes too many blood cells. It is also sometimes just called polycythaemia (PV). There is an abnormally high number of red blood cells in your blood. Also, about 1 in 10 people with PRV develop gout. Mielofibrosi There is a chance that, after about 15.
Patient information leaflet
Colangite sclerosante primitiva
Colangite sclerosante primitiva is an uncommon condition affecting the bile ducts and liver. Inflammation and scarring of the bile ducts can lead to liver damage and cirrhosis - a condition where normal liver tissue è replaced by scar tissue (fibrosis). Different treatments are available to control symptoms that may develop and also to manage any.
Patient information leaflet
Ittero
Conditions affecting the globuli rossi Various conditions cause an increased rate of breakdown of red blood cells. As a result, there is more bilirubin made than usual (most bilirubin is made from the breakdown of haemoglobin within red blood cells) which then circulates in the blood. The liver cells are unable to keep pace in processing the.
Ulteriori letture e riferimenti
- Primary myelofibrosis; MedlinePlus, National Library of Medicine.
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- Saint-Martin C, Leroy G, Delhommeau F, Panelatti G, Dupont S, James C, Plo I, Bordessoule D, Chomienne C, Delannoy A, Devidas A, Gardembas-Pain M, Isnard F, Plumelle Y, Bernard O, Vainchenker W, Najman A, Bellanne-Chantelot C; French Group of Familial Myeloproliferative Disorders. Analysis of the ten-eleven translocation 2 (TET2) gene in familial myeloproliferative neoplasms. Blood. 2009 Aug 20;114(8):1628-32. doi: 10.1182/blood-2009-01-197525. Epub 2009 Jun 29.
- Tefferi A, Pardanani A, Lim KH, Abdel-Wahab O, Lasho TL, Patel J, Gangat N, Finke CM, Schwager S, Mullally A, Li CY, Hanson CA, Mesa R, Bernard O, Delhommeau F, Vainchenker W, Gilliland DG, Levine RL. TET2 mutations and their clinical correlates in polycythemia vera, essential thrombocythemia and myelofibrosis. Leukemia. 2009 May;23(5):905-11. doi: 10.1038/leu.2009.47. Epub 2009 Mar 5.
- Tefferi A. Novel mutations and their functional and clinical relevance in myeloproliferative neoplasms: JAK2, MPL, TET2, ASXL1, CBL, IDH and IKZF1. Leukemia. 2010 Jun;24(6):1128-38. doi: 10.1038/leu.2010.69. Epub 2010 Apr 29.
- Tefferi A. Pathogenesis of myelofibrosis with myeloid metaplasia. J Clin Oncol. 2005 Nov 20;23(33):8520-30. doi: 10.1200/JCO.2004.00.9316.
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