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Myelofibrosis is a cancerous change in the bone marrow that causes fibrosis of this tissue. This causes serious disturbances in the blood composition, which is associated with serious health complications. Worse, the disease is extremely difficult to treat and diagnose. Fortunately, it belongs to the less common neoplastic changes in the bone marrow.
What is myelofibrosis?
The exact causes of this type of bone marrow cancer are unknown. It is known that the bone marrow cells mutate genetically and they keep multiplying, causing the disease to progress. The result of this process is an overgrowth of the connective tissue consisting of collagen, i.e. fibrosis. As a result, hematopoietic cells in the marrow are displaced by collagen and are unable to achieve the efficiency necessary for the proper functioning of the body. Leukocytes can be low in myelofibrosis, but sometimes too many of them multiply. Red blood cells are not the correct shape and size. The platelets are often over-abundant, which facilitates the formation of blood clots. Other cells often enter the blood in an underdeveloped form.
Myelofibrosis – diagnosis and symptoms
The diagnosis of myelofibrosis is extremely difficult due to the fact that the disease at the initial stage gives non-specific symptoms, that is, those that occur in many different diseases. The most common ones include:
- weakening of the overall efficiency of the body, chronic fatigue, shortness of breath after exercise,
- night sweats
- flatulence, a feeling of heaviness in the abdomen, lack of appetite,
- palpitations, fast heart rate.
Sometimes there is pain in the legs caused by myelofibrosis and their swelling is related to the tightening of the veins. At a later stage of the disease, the so-called extramedullary hematopoiesis when other organs try to replace the bone marrow in blood production. Most often, this function is taken over by the spleen, which results in its significant enlargement – in a healthy person, the spleen weighs about 150 grams, and in extreme cases it grows up to several kilograms in people with myelofibrosis.
The development of myelofibrosis leads to a weakening of the body’s immunity and susceptibility to infections, damage to internal organs, and in acute cases, the disease transforms into acute myeloid leukemia and the patient dies.
Unfortunately, it is not uncommon for myelofibrosis to go undiagnosed until the advanced stage of the disease due to the aforementioned nonspecific symptoms. Patients are sometimes treated for anemia, which causes very similar symptoms, often being a symptom of myelofibrosis itself. Sometimes this early stage lasts several years, in other cases the disease progresses much faster.
If myelofibrosis is suspected, the doctor first examines the abdominal cavity to detect any abnormalities in the size of the spleen. This can be done manually or by ultrasound. The final diagnosis is trepanobiopsy, which is a bone marrow sample, usually from the hip bone.
The incidence of myelofibrosis
Myelofibrosis is considered the rarest bone marrow cancer. Statistically speaking, it is 1,5 cases for every 100 people. It usually affects people over 60, although in recent years there has been a higher incidence of myelofibrosis among younger people.
Myelofibrosis – treatment and prognosis
Unfortunately, it must be honestly said that the prognosis for this type of bone marrow cancer is not good. If the treatment is started too late, the patient has no more than a few years of life. Even if individual internal organs can be kept relatively well functioning, there is a high risk of death from internal hemorrhage or infection, to which susceptibility increases with the development of myelofibrosis.
The only way to fully cure myelofibrosis is through bone marrow transplantation. However, it is possible only at an early stage of cancer development, and of course also requires finding a bone marrow donor. In other cases, a variety of methods are used to limit the growth of a tumor and its effects on the body. Radiotherapy, chemotherapy, blood transfusions are used, and a number of different preparations are given that affect individual body systems that are not working properly. Often times, you may need a splenectomy, which is surgery to remove an enlarged spleen.