Erythremia - what is it? Stage eritremii

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2018-03-28 19:58:21

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Erythremia – is one of the types of blood disorders that is characterized by proliferation (proliferation) of red blood cells and a marked increase in the number of other cells (leukocytes, platelets). Is a chronic, often benign, the disease quite rare. However, the transformation of benign to malignant leukemia.erythremia - aErythremia – a disease that a year is sick about 4 people from 100 million people. It does not depend on gender, but usually diagnosed in people older and older. Although the reported cases of the disease and at a younger age, often it's women.

Erythremia (code ICD-10 - С94.1) has a chronic course.

Blood Cells and the mechanism of disease

The Mechanism eritremii develops as follows.

Erythrocytes-red blood cells, whose main function-is the transfer of oxygen to all cells of our body. They are produced in the bone marrow, spleen and liver. Bone marrow is found in long bones, vertebrae, ribs, skull bones and sternum. 96% red blood cells filled with hemoglobin, which performs a respiratory function. Red color gives them iron. Erythrocytes are formed with the help of stem cells. Their distinctive ability – ability to share (replicate) into any other cell.

Bones is yellow marrow, which is represented by adipose tissue. He begins to produce blood elements only in extreme conditions when other sources of hematopoiesis do not perform their functions.

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Erythremia (ICD-10 - international classification of diseases in which this disease has code With 94.1) – the disease, the pathogenesis of which has not been studied. For reasons that still remain unclear to doctors, the body begins to vigorously develop red blood cells, which cease to fit in the bloodstream, increasing blood viscosity and begin to form blood clots. Over time, increases hypoxia (oxygen starvation). Cells do not receive proper nutrition, there is a failure in the overall system of the body.

Some interesting facts about eritremii

  1. Erythremia – this is the most benign disease of leukemia. That is a long time she was passive enough and later leads to complications.
  2. It may be asymptomatic and not know about themselves for many years.
  3. Despite the increased platelet level, patients are prone to profuse bleeding.
  4. Erythremia has a family history, so if the family is the patient, the risk of appearance of one of my relatives increases.

Causes of erythremia

Like other types of leukemia, erythremia still not fully understood, and the reasons for its occurrence have not been identified. However, articulated the factors that have to its creation:

  1. Genetic predisposition.
  2. Toxic substances trapped in the body.
  3. Ionizing radiation.

Genetic predisposition

Currently it is not known what gene mutations cause the appearance of erythremia, but found that in the family the disease is quite often repeated in the next generations. The likelihood of developing disease is increased significantly if a person is suffering:

  • Down's syndrome (disruption of the face and neck and developmental delays);
  • Klinefelter syndrome (disproportionate figure and possible developmental delays);
  • Bloom syndrome (low growth, pigmentation on face and his disproportionate development, predisposition to cancer);
  • Marfan syndrome (a developmental disorder of connective tissue).

Predisposition to eritremii is due to the fact that the genetic cell apparatus (including apparatus blood) is unstable, so the person becomes susceptible to external negative effects-toxins, radiation.erythremia blood test indicators

Radiation

Even x-rays and gamma rays partially absorbed by the body, affecting the genetic cells. They can die or mutate.

However, the most powerful radioactive radiation are people who treat cancer with chemotherapy and those who were in the epicenter of power plants or atomic bombs.

Toxic substances

When ingested, they can cause genetic mutation of cells. These substances are called chemical mutagens. After numerous studies, scientists have found that patients with erythema in contact with these substances before the development of the disease. Such substances include:

  • Benzene (found in petrol and many chemical solvents);
  • Antibacterial drugs (in particular, "Chloramphenicol");
  • Cytotoxic drugs (anticancer).

Stage eritremii and their symptoms

Erythremia stage has the following: primary, extended and terminal. Each of them has its symptoms. Initial stage may last for decades, not manifesting any serious symptoms. Minor symptoms the patients usually blamed on other, less serious diseases. Even doctors often do not pay enough attention to the imperfect analysis of the blood.

If there is initial erythema, blood tests have a moderate deviation from the norm.

The Initial stage is also characterized by rapidfatigue, dizziness, noise in the ears. The patient sleeps badly, feels cold limbs, edema of hands and feet. A decrease in mental performance. No external manifestations of the disease yet. Headache is not considered a specific symptom of the disease, but it occurs at the initial stage due to poor circulation in the brain. For this reason, reduced vision, attention and intelligence.chronic erythremiaWhen the diagnosis of "erythremia" the symptoms of the disease in the second stage have their own characteristics – the gums bleed, become visible bruise. On the lower leg may appear dark spots (symptoms of DVT), and even sores. Increase the organs in which the formation of red blood cells-the spleen and liver. Due to the development of the disease the lymph nodes to swell.

The Second stage eritremii can last about 10 years. Weight is markedly reduced. The skin takes on a cherry tone (often arms and legs), soft sky changes color, solid – retains its previous color. The patient concerned itching, which is amplified after bathing in warm or hot water. On the body become visible swollen veins from too much blood, especially in the neck. The eyes seemed bloodshot, and because the erythema, the symptoms of which are quite explicit in the second stage, facilitates the flow of blood to the vessels of the eye.

Due to poor circulation in the capillaries is manifested the pain and burning sensation in the fingers and toes. In advanced cases, become visible titanicheskie spots on them.

Have the bone and epigastric pain. The pain in the joints of a gouty nature associated with an excess of uric acid. Generally, kidney function is disturbed, is often diagnosed pyelonephritis and found uratovye kidney stones.

The nervous system Suffers. The patient is nervous, his mood is unstable, characterized by tearfulness and changes frequently.

Blood Viscosity increases, which leads to the appearance of blood clots in different vascular areas. There is also the risk of varicose veins development.

Attention! Patients can also suffer from hypertension and open ulcers in the duodenum. It is associated with reduced protective forces of an organism and the increased number of Helicobacter pylori-this bacteria causes ulcers.

The Third stage eritremii is characterized by pale skin, frequent fainting, weakness and lethargy. Observed prolonged bleeding during minimal injuries, aplastic anemia due to the decrease in the level of hemoglobin in the blood.

Third stage eritremii: symptoms and complications

Erythremia-a blood disorder that in the third stage becomes aggressive. At this stage, the bone marrow cells are exposed to fibrosis. He can no longer produce red blood cells, so the rules are formed elements of blood are falling, sometimes to the critical values. In the brain appear foci of softening, starts liver fibrosis. In the gallbladder is thick, viscous bile and pigment stones. The result is liver cirrhosis and blockage of blood vessels.

Previously, it increased blood clots were the cause of death of patients with erythema. Blood vessels undergo changes, there is a blockage of the veins in the blood vessels of the brain, spleen, heart and feet. Occurs occlusive disease-blockage of blood vessels of the feet with the risk of their total restriction.

The kidneys are Affected. Due to the increase of uric acid patients suffer from joint pain gouty character.

Attention! In eritremii feet and hands often change the hue. The patient is prone to bronchitis and colds.

Disease

Erythremia – it is a slow disease. The disease begins slowly, its beginning is gradual and subtle in nature. Patients sometimes live for decades, not paying attention to minor symptoms. However, in more serious cases, blood clots death may occur within 4-5 years.

Along with the erythema increased spleen. Isolated form with cirrhosis of the liver and lesion of the diencephalon. In the course of the disease may be involved with allergic and infectious complications, sometimes patients can not tolerate certain drugs, suffering from urticaria and other skin diseases. The disease is complicated by concomitant conditions, because the diagnosis is usually made for elderly people.erythremia symptoms

Posteriormente myelofibrosis – rebirth of the disease of benign course and malignant. The state is a natural patients who survived to this period. In this case, the doctors admit that erythremia acquired neoplastic in nature.

It is important to properly diagnose the erythremia and assign appropriate treatment. Diagnosis includes a wide range of studies.

Blood in eritremii

When the diagnosis of "erythremia" analysis of blood, indicators of which are very important for diagnosis, is done in the first place. First of all, it is the General analysis of blood. It is the deviation from the norm to become the first disturbing call. Increased number of red blood cells. Initially, it is not critical, however, with the development of the disease is increasing, and in the last stage falls. The normal level of red blood cells in the blood of women is between 3.5 to 4.7, and men – from 4 to 5.

If diagnosed erythremia, analysis of blood, indicators of which indicateto increase red blood cells, says about the appropriate increase in hemoglobin. The hematocrit indicates the ability of the blood to carry oxygen is increased to 60-80 %. Color index is not changed, but in the third stage can be anything – normal, high or low. Platelets and white blood cells increased. Leukocytes grow up several times, and sometimes more. The production of eosinophils (sometimes together with basophils) increases. Increased platelets. The erythrocyte sedimentation rate – not above 2 mm/hour.erythremia stage

Alas, a common blood test, though important in the initial diagnosis, uninformative, and only on the basis of the diagnosis is not put.

Other studies in eritremii

  • Biochemical analysis of blood. Its main goal – to determine the amount of iron in the blood level and liver function tests-AST and ALT. They are released from the liver in the destruction of its cells. Determining the quantity of bilirubin indicates the severity of the process of destruction of red blood cells.
  • Bone marrow Puncture. The analysis is taken using the needle that is inserted into the skin in the region of the periosteum. The method indicates the status of the hematopoietic cells in the bone marrow. The study indicates the number of cells in the bone marrow, the presence of cancer cells and fibrosis (growth of connective tissue).
  • When the diagnosis of "chronic erythremia" is also doing laboratory markers and abdominal ultrasound. Ultrasound reveals an overflow of blood, enlarged liver and spleen and foci of fibrosis within them.
  • Doppler indicates the speed of blood flow and helps to detect blood clots.

Treatment of erythremia

It Should be noted that the erythema is often progresses slowly and has a benign course. First and foremost, doctors recommend lifestyle changes – more fresh air, to walk, to positive emotions (endocrinotherapy sometimes reaches amazing results). Foods that contain large amounts of iron and vitamin C, should be excluded.

The first stage of the disease the main goal in diagnosis of "erythremia" - blood counts reduce to normal: hemoglobin up to 150-160, and hematocrit – 45-46. It is also important to negate complications caused by the disease – circulatory disorders, pain in the fingers, etc.erythremia blood counts

To Normalize hematocrit hemoglobin helps the bloodletting, which in this case is used to this day. However, this procedure is done in the framework of emergency aid, because it stimulates the bone marrow and the function of thrombopoiesis (the formation of platelets). There is also a procedure called erythrocytapheresis, which is to cleanse the blood of erythrocytes. The blood plasma remains.

Medication

Cytostatics are drugs of anticancer of nature, which is used for complications eritremii – ulcers, thrombosis, blood circulation disorders of the brain arising in the second stage of the disease. "Mielosan", "Busulfan" hydroxyurea, "Imifos," the radioactive phosphorus. The latter is considered effective because it accumulates in bones and inhibits the function of the brain.

Corticosteroids are prescribed in hemolytic anemia of autoimmune origin. Especially popular is "Prednisolone". If the treatment does not give the appropriate results, prescribe surgery to remove the spleen.

To avoid iron deficiency can be prescribed iron tablets – "Hemofer", "Totem", "Drops".erythremia treatment

As necessary, designate the following groups of medicines:

  1. Antihistamines.
  2. Reduce blood pressure.
  3. Anticoagulants (blood thinners).
  4. Medications.

Erythremia treatment which is often carried out in a hospital is a serious disease that causes many complications. It is important to identify it and start treatment.


Article in other languages:

AR: https://tostpost.com/ar/health/6281-erythremia---eritremii.html

BE: https://tostpost.com/be/zdaro-e/11209-eritremiya---geta-shto-takoe-stady-eritremii.html

DE: https://tostpost.com/de/gesundheit/11210-eritremia---was-ist-das-phase-eritremii.html

ES: https://tostpost.com/es/la-salud/11214-eritremiya-es-lo-que-es-la-etapa-de-eritremii.html

HI: https://tostpost.com/hi/health/6285-erythremia---eritremii.html

JA: https://tostpost.com/ja/health/6283-erythremia-eritremii.html

KK: https://tostpost.com/kk/densauly/11211-eritremiya---b-l-ne-satysynda-eritremiya.html

PL: https://tostpost.com/pl/zdrowie/11204-eritremiya---to-co-to-jest-etapie-eritremia.html

PT: https://tostpost.com/pt/sa-de/11200-eritremiya---que-isso-a-fase-de-eritremii.html

TR: https://tostpost.com/tr/sa-l-k/11215-eritremia---bu-nedir-sahne-eritremia.html

UK: https://tostpost.com/uk/zdorov-ya/11207-eritrem-ya---ce-scho-take-stad-eritrem.html

ZH: https://tostpost.com/zh/health/6804-erythremia--eritremii.html






Alin Trodden - author of the article, editor
"Hi, I'm Alin Trodden. I write texts, read books, and look for impressions. And I'm not bad at telling you about it. I am always happy to participate in interesting projects."

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