Myeloproliferative syndromes: types and causes

Most people are familiar with the term leukemia. You know that this is a very aggressive and dangerous type of cancer in which cancer cells are found in the blood, affecting infants to the elderly and probably also originating in the marrow I mean. It is one of the best known myeloproliferative syndromes. But it is not unique.

In this article we will briefly describe what are myeloproliferative syndromes and we will indicate some of the most frequent.

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Myeloproliferative syndrome: what are they?

Myeloproliferative syndromes are a group of syndromes characterized by the presence of a excessive and accelerated growth and reproduction of one or more types of blood cells; specifically of myeloid lines. In other words, there is an excess of some type of blood cells.

These kinds of problems are caused by excessive production of stem cells that will end up producing red blood cells, white blood cells or platelets. In adults these cells are produced only by the bone marrow, although during development the spleen and liver also have the capacity to produce them. These two organs tend to grow in these diseases because the excessive presence of myeloid in blood causes them to recover this function, which in turn causes an even greater increase in the number of cells sanguine.

Yes ok symptoms may vary depending on myeloproliferative syndromes of which we are talking, generally coincide in the appearance of typical anemia problems, such as the presence of weakness and physical and mental fatigue. Gastrointestinal and respiratory problems, loss of weight and appetite, fainting, and vascular problems are also common.

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Why are they produced?

The causes of these diseases are associated with mutations in the Jak2 gene on chromosome 9, which causes erythropoietic stimulating factor or EPO acts continuously (in subjects without these mutations, EPO only acts when necessary).

In most cases these mutations are not hereditary but acquired. It is speculated that may influence the presence of chemicals, radiation exposure or poisoning.

Some of the main myeloproliferative syndromes

Although with the passage of time new syndromes and variants of these are discovered, in general myeloproliferative syndromes are classified into four types, differentiated largely by the type of blood cells that proliferate.

1. chronic myeloid leukemia

The disease discussed in the introduction is one of the different existing leukemias and one of the best-known myeloproliferative syndromes. This type of leukemia is caused by the excessive proliferation of a type of white blood cells. known as a granulocyte.

Fatigue is common and asthenia, bone pain, infections and bleeding. In addition to this, it will produce different symptoms depending on the organs where the cells infiltrate.

It usually appears in three phases: chronic, in which asthenia and loss of that appear due to viscosity loss of blood, loss of appetite, kidney failure, and abdominal pain (at which time it is usually be diagnosed); the accelerated, in which problems such as fever, anemia, infections and thrombosis arise (this being the phase in which bone marrow transplantation is usually used); and the blastic, in which symptoms worsen and the level of cancer cells exceeds twenty percent. Chemotherapy and radiotherapy are often used, along with other medications that allow fighting cancer.

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2. polycythemia vera

Polycythemia vera is one of the disorders classified within the myeloproliferative syndromes. In polycythemia vera, the bone marrow cells cause the appearance of erythrocytosis or the presence of excessive amount of red blood cells (the cells that carry oxygen and nutrients to the rest of the body's structures) in the blood,. More than the number of blood cells, what marks the onset of this disease is the amount of hemoglobin that is transported A higher number of white blood cells and platelets is also seen.

The blood becomes thicker and more viscous, which can cause occlusions and thrombosis, as well as unexpected bleeding. Typical symptoms include flushing, congestion, weakness, itching, and pain of varying intensity (especially in the abdomen), dizziness, and even vision problems. One of the most specific symptoms is generalized itching throughout the body. Pain with redness of the extremities is also common, caused by occlusion and circulation difficulties in small blood vessels. Uric acid also tends to skyrocket.

Although it is serious, chronic and requires treatment and control of possible complications, this disease does not usually shorten the life expectancy of those who suffer from it if it is treated correctly.

3. essential thrombocythemia

This syndrome is characterized by the excessive production and presence of platelets in the blood. These cells mainly fulfill the function of blood coagulation and are related to the ability to heal wounds.

The main problems that this disease can cause is the provocation of thrombosis and bleeding in the subject, which could have serious repercussions on health and even end life of the subject if they occur in the brain or heart. It can lead to myelofibrosis, much more complex.

In general, it is considered that this problem does not necessarily shorten the life of those who suffer from it, although they should periodic controls to control the level of platelets and, if necessary, reduce it by means of treatment.

4. myelofibrosis

Myelofibrosis is a disorder. It can be primary if it appears by itself or secondary if it is derived from another disease.

Myelofibrosis is one of the most complex myeloproliferative syndromes.. This time, the stem cells in the bone marrow that should produce the blood cells over-generate them in such a way that, in the long run, increases are generated in the fibers of the marrow that end up causing the growth of a kind of scar tissue that occupies the place of the marrow. Blood cells, likewise, end up immature and unable to perform their functions in a normative manner.

The main symptoms are due to anemia caused by immature blood cells, the excessive growth of the spleen caused by this and alterations in metabolism. Thus, it is common for fatigue, asthenia, sweating, abdominal pain, diarrhea, weight loss and edema to appear.

Myelofibrosis is a serious illness in which anemia ends up appearing and even a drastic reduction in the number of functional platelets that can cause serious bleeding. In some cases it can lead to leukemia.

Bibliographic references:

• Hernandez, L.; Besses, C. and Cervantes, F. (2015). Myeloproliferative syndromes. General information for the patient. AEAL Explains. Madrid.