Osteomyelofibrosis: symptoms and treatment

Osteomyelophybrosis is an ailment that is based on bone marrow damage. This disease belongs to the leukemia group. During the progression of the disease, the connective tissue in the tubular bones proliferate, and the hematopoietic tissue is replaced by fibrous and bone tissue. In most cases, osteomyelophybrosis affects people from fifty to seventy years old.



Etiology

Osteomyelophybrosis can appear and develop due to prolonged exposure to toxic substances and / or ionizing radiation. These factors cause many diseases, so specialists do not consider them the only and the main reasons for the appearance of osteomyelophybrosis. In most cases, this ailment is a complication of the following diseases:


  • myeloid leukemia. This is a tumor disease of the blood, which is characterized by a constant and uncontrolled reproduction and an increase in all the germ cells. The danger of the ailment lies in the fact that young, inferior cells very quickly mature to adult forms. Myeloleukemia has three stages of development. The chronic phase of an ailment is characterized by the fact that a person is sometimes disturbed by minor symptoms. General weakness, malaise, decreased appetite and sweating at night the patient justifies the usual overwork. It is rather difficult to detect myeloid leukemia at a chronic stage of development, and in most cases it happens by chance in the process of performing a general blood test. This phase lasts about four years, and if a person does not take any measures, it flows into the stage of acceleration. At this time, the pathological process in the patient’s body is activated, and the number of immature white blood cells is rapidly increasing. It is the phase of acceleration of myeloleukemia and is considered the beginning of osteomyelophybrosis. If the disease is not stopped, then the terminal stage of its development will soon come. This is the final phase of myeloid leukemia, during which the red bone marrow is almost completely replaced by poor-quality cells. As a rule, it is at the terminal stage that a person dies.
  • ailments of the immune system. Decreased immunity, various dysfunctions of the immune system are the cause of a number of diseases. Many of these diseases can cause osteomyelophybrosis.

Pathogenesis

During the development of the disease affects the bone marrow, connective tissue grows, and the hemopoietic tissue is replaced by fibrous and bone tissue. In addition, the patient has an increase in spleen due to the fact that it is this body that takes an active part in the blood. So the human body tries to compensate for the decrease in the production of blood cells in the bone marrow.

Symptoms

Disturbance of hematopoiesis, which is characterized by the initial stage of development of osteomyelophybrosis, leads to the appearance of clinical signs of the disease:


  1. general weakness;
  2. fast fatigue;
  3. increase in body temperature;
  4. reducing resistance to infectious ailments
  5. pale skin;
  6. gradual weight loss
  7. rapid saturation, dull pain in the left abdominal region, the presence of a tumor-like formation, which is easily palpated. All these symptoms are due to an increase in the spleen;
  8. bleeding or the occurrence of thrombi. These symptoms are not very common and are due to a disorder in the function of platelets and white blood cells;
  9. headaches, distracted attention, dizziness, blurred vision, dyspnea. This is also not very common signs, due to the increased coagulability of blood.

Diagnostics

If the first signs of the disease appear, see a doctor. If osteomyelofibrosis has not yet passed into the accelerating phase, then the patient can be saved. Reception at the doctor-hematologist passes as follows:


  • Talk with the patient. The specialist will ask you about your general state of health and the timing of the onset of symptoms. In addition, the doctor can ask questions about existing chronic diseases;
  • probing of lymph nodes
  • Abdominal palpation. So the hematologist learns how much the spleen is enlarged;
  • direction for additional research. The doctor can not correctly diagnose and prescribe the necessary treatment without studying the results of a blood test, ultrasound examination, bone marrow puncture, cytochemical and cytogenetic studies.

Treatment

To date, there is no generally accepted treatment of osteomyelophybrosis. The choice of therapy is influenced by the general condition, age and patient’s illnesses, as well as the development phase of the disease.


  1. drug treatment. Pharmacological agents suppress the growth of malignant cells and contribute to the process of their natural death. Often, patients are prescribed glivec, which is released in the form of tablets. Side effects of this drug is difficult to assess because of the fact that the patient already has pronounced disorders of various organs. Nevertheless, from time to time, nausea, vomiting, loose stools and muscle cramps are observed;
  2. Surgical intervention. If a person suffers from severe discomfort in the abdomen or has thrombocytopenia, the doctor decides to remove the spleen. This operation facilitates the course of osteomyelophybrosis and improves the patient’s well-being, in fact, together with the spleen, a large number of poor-quality cells are removed from the body. In addition, after surgical intervention, the effectiveness of drug treatment is markedly increased,
  3. bone marrow transplant. Of course, this method gives a positive result, but experts rarely use it. The whole point is that bone marrow transplantation can be performed only for patients under 50 years old, who do not suffer from dangerous chronic diseases. The body of an elderly person may simply not suffer such interference. According to statistics, the survival rate of people over 50 years after bone marrow transplantation is about 14%.




If a person does not take any measures, or none of the proposed methods of treatment does not suit him, the prognosis is disappointing. Osteomyelophybrosis gradually worsens the functioning of the immune system, which leads to severe complications. As a rule, in such cases, the life expectancy of the patient is from six to eight years.