Myeloproliferative syndromes are a group of neoplastic diseases of the hematopoietic system in which there is an overproduction of one or more types of peripheral blood cells. Which blood cancers are included in this group? What symptoms might indicate a myeloproliferative disorder?

Myeloproliferative syndrome (MPS) is a very diverse group of diseases that differ in etiology and clinical picture. The myeloproliferative syndromes include the following diseases:

  • chronic myeloid leukemia
  • polycythemia real
  • essential thrombocythemia
  • chronic eosinophilic leukemia
  • myelofibrosis
  • mastocytosis
  • chronic neutrophilic leukemia
  • chronic myelomonocytic leukemia
  • atypical form of chronic myeloid leukemia

However, these very different diseases have several common features, including symptoms such as:

  • splenomegaly, which is quite characteristic of most hematological diseases
  • increase in uric acid concentration in the blood, which may result, among others, from increased "cell turnover"
  • increase in the number of all cells in the initial stage of the disease
  • increase in basophil concentration
  • tendency to fibrosis and hardening of the bone marrow
  • extramedullary outbreaks of hematopoiesis
  • thrombosis tendency
  • good efficacy of interferon α treatment
  • presence of the V617F mutation in the JAK2 gene

Below is a shortened specificity of the above-mentioned myeloproliferative diseases.

Chronic myeloid leukemia

This myeloproliferative disease accounts for approximately 15 percent of all adult leukemias, and mostly affects people between the ages of 30 and 50.

In almost half of the patients it is detected accidentally during the routine blood count, which shows leukocytosis with an increased percentage of neutrophils and basophils, additionally rejuvenated granulocytic line.

The only certain etiological factor is exposure to ionizing radiation. With a significantly increased number of leukocytes, their local "retention" in various parts of the body, which leads to "clogging" of the smallest vesselsblood vessels. This phenomenon is called leukostasis.

Leukostasis may be manifested by dizziness and headaches, disturbances in consciousness, vision, or symptoms of hypoxia.

Symptoms characteristic of anemia are also often present, such as pale skin and rapid fatigue.

As in other myeloproliferative syndromes, splenomegaly is observed.

This myeloproliferative disorder is characterized by the presence of the Philadelphia chromosome, which is present in up to 95 percent of chronic myeloid leukemia.

The natural course of chronic myeloid leukemia can be divided into 3 stages: chronic phase, acceleration phase and blast crisis phase.

The most important thing in the treatment of chronic myeloid leukemia is to stop its development in the chronic stage, which significantly increases the patient's chance of a favorable prognosis.

Several drugs are used in the treatment of this myeloproliferative syndrome, and the success of the therapy is achieved when we achieve hematological, cytogenetic and molecular remission.

Czerwienica real

This myeloproliferative syndrome occurs most often around the sixth-seventh decade of life, and affects the male sex more often. The causes of polycythemia vera are not fully understood.

It is characterized by a significant increase in the number of red blood cells in the peripheral blood, often accompanied by leukocytosis and thrombocytosis.

True polycythemia, apart from the features typical of all myeloproliferative syndromes, is also characterized by:

  • redness and redness of the face, conjunctiva, optic disc, hands and feet
  • itching, which is aggravated by hot baths
  • hypertension
  • symptoms typical of gastric ulcer and duodenal ulcer
  • gout
  • headaches and dizziness
  • tinnitus
  • visual impairment
  • erythromelalgia, that is, paroxysmal pains in the hands and feet, accompanied by their redness

Treatment of this myeloproliferative syndrome largely depends on the clinical picture of the patient and the course of the disease. Sometimes regular bleeding is enough.

Often, if there are no contraindications, acetylsalicylic acid is used as a thrombosis prophylaxis.

In addition, the drugs that are used in the treatment of myeloproliferative syndrome such as polycythemia vera are: hydroxycarbamide, interferon α, busulfan, allopurinol.

essential thrombocythemia

In this myeloproliferative syndrome it comes toincrease the number of platelets and increase the production of megakaryocytes in the bone marrow.

Essential thrombocythemia mainly affects people between the ages of 50 and 60.

The symptoms of thrombocythemia depend on the number of platelets and possible disturbances in their functioning. The most common symptoms are thrombosis of small and large blood vessels.

Sometimes a hemorrhagic diathesis occurs as a result of abnormal platelet function, and then we can observe bleeding from the mucous membranes or from the gastrointestinal tract.

In the treatment of this myeloproliferative syndrome, medications similar to those in the treatment of, among others, polycythemia vera can be used.

Myelofibrosis

Myelofibrosis is a myeloproliferative syndrome characterized by aplasia and bone marrow fibrosis followed by the formation of extramedullary hematopoiesis.

The symptoms of myelofibrosis are similar to those of other myeloproliferative syndromes, such as splenomegaly, weight loss, weakness, fever, bleeding, and tendency to infections.

Mastocytosis

The myeloproliferative syndrome, which is mastocytosis, is based on the excessive production of mast cells, i.e. mast cells. These are cells that belong to the immune system and are involved, among others, in allergic reactions or anaphylactic shock.

Therefore, the symptoms of mastocytosis will be similar to those that appear in the course of anaphylaxis, such as shortness of breath, edema, hives and other skin changes, abdominal pain or diarrhea.

Depending on where mast cells accumulate, we can distinguish between cutaneous and systemic mastocytosis. In the diagnosis of this myeloproliferative syndrome, in addition to typical laboratory tests, biopsy of the skin or other organ affected by the disease is also used.

Chronic Neutrophilic Leukemia

Chronic neutrophilic leukemia is a myeloproliferative disorder in which there is excessive proliferation of mature neutrophils. Symptoms may include, for example, hepato- or splenomegaly, bleeding of various degrees and often gout. In the treatment of this myelopoliferative syndrome, drugs such as hydroxycarbamide, interferon α or busulfan are used.

Chronic Myelomonocytic Leukemia

This myeloproliferative syndrome develops as a result of chronic monocytosis. There are two types of chronic myelomonocytic leukemia, more specifically the myelodysplastic and myeloproliferative subtypes. This myeloproliferative syndrome is diagnosed more often in the male gender.

Tosymptoms of chronic myelomonocytic leukemia are typical symptoms of most hematological diseases, as well as symptoms resulting from deficiencies of individual cell lines, such as pale skin, greater susceptibility to infection and a tendency to prolonged bleeding. The only cure for this myeloproliferative syndrome is a bone marrow transplant.

Category:

Oncology