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Myelofibrosis (MF) is one of the myeloproliferative neoplasm (MPN) where there are too many fibroblasts in the bone marrow.
Myelofibrosis (MF) is a disorder of the bone marrow. MF is considered a rare disease as it affects fewer than 1-2 people in every 100,000 per year.
There are two types of Myelofibrosis:
ET, PV and MF are all closely related diseases that come under the category of myeloproliferative neoplasms (MPNs).
As times goes on, we are learning more about MF. However, what causes MF is not fully understood.
One of the features of the disease that we do know is dysregulated signaling in haematopoeitic stem cells (cells in the bone marrow that make your blood cells). One of the signaling pathways that has been found to be frequently involved (in about 50-60% of patients) is called ‘JAK-STAT,’ which means there is a change in JAK2.
However, even MF patients who do not have this JAK2 mutation may still have dysregulated ‘JAK-STAT’ signaling that underlies their disease.
A further 25% of patients have a mutation in a gene called calreticulin-R (CAL-R) and about 5% of patients have mutation of a gene called MPL.
New genetic mutations are still being discovered and it is likely that, in most patients, the disease is caused by a combination of these mutations.
In someone with MF, abnormal stem cells take over the bone marrow, leading to chronic inflammation and fibrosis (scarring). Through the bone marrow not being able to make normal blood cells, the spleen and liver (and sometimes other organs) try to compensate by producing red blood cells. As a result, the spleen can become enlarged.
Enlargement of the spleen may cause abdominal pain, discomfort, loss of appetite and a feeling of filling up quickly during meals.
Due to the lack of blood cells being produced in the bone marrow, there are a number of conditions that are common in MF patients:
The most common symptoms of MF include:
Diagnosis will usually be made following a number of tests, these may include:
Your personal situation, health history and the ways in which you respond to treatment can all affect your prognosis.
Prognostic scoring systems are used by doctors, either at the time that the diagnosis of MF has been made (International Prognostic Scoring System, IPSS) or at later points during the course of the disease (Dynamic International Prognostic Scoring System, DIPSS or DIPSS Plus). Generally, these systems allow MF to be categorized as ‘low risk’, ‘intermediate-1’, ‘intermediate-2’ or ‘high risk’.
Certain factors may influence prognosis and will be used by your doctor to calculate your score:
The majority of treatments for MF are aimed at managing symptoms and reducing complications, so that your quality of life is better.
Due to the high risk of life-threatening side effects and the risk of new stem cells reacting against your body’s healthy tissues, transplants are not commonly recommended. The first step, known as ‘conditioning’, involves high levels of chemotherapy or radiation therapy.
In general terms, all other treatments can be divided into two main groups of treatments:
1. Treatments that are used to reduce the size of the spleen and to improve constitutional symptoms
2. Treatments that are used primarily to treat anaemia and to improve low blood counts:
Many alternative treatments for MF are being actively investigated, such as other JAK inhibitors including inhibitors of a pathway known as the Hedgehog pathway, telomerase inhibitors and histone deacetylase inhibitors. In years to come, it is likely that there will be many more treatment options available to patients.