Erythremia is a malignant blood pathology, accompanied by intense myeloproliferation, resulting in the appearance of a large number of erythrocytes in the bloodstream, as well as some other cells. Erythremia is also called true polycythemia. In other words, it is chronic leukemia.
The blood cells formed in an overabundance have a normal shape, structure. Due to the fact that the number of them increases, the viscosity increases, the blood flow slows down significantly, thrombi begins to develop. All this causes problems with blood supply, leads to hypoxia, which increases with time. The first time about illness in the late nineteenth century, Vakez spoke, and already in the first five years of the twentieth century, Osler told about the mechanism of the appearance of this pathology of blood. He also defined erythremia as a separate nosology.
Despite the fact that about erythremia is known for almost a century and a half, it is still poorly understood, the reliable causes of the occurrence are unknown.
Erythemy of the ICD (International Classification of Diseases) - D45. Some researchers concluded during the epidemiological monitoring that erythremia has a relationship with the processes of transformation in stem cells. They observed a mutation of tyrosine kinase (JAK2), in which phenylalanine replaced valine in six hundred and seventeenth position. Such anomaly is a companion of many blood diseases, but with erythremia occurs especially often.
It is believed that there is a family predisposition to the disease. So, if close relatives were ill with erythremia, the chance to get this disease in the future increases. There are also some patterns of the origin of this pathology. Erythremia is mainly affected by people aged (sixty to eighty years), but there are isolated cases when it develops in children, young people. In young patients, erythremia is very difficult. Men are 1.5 times more likely to suffer from ailment, but among the rare cases of morbidity among young people, the female sex prevails.
Among all blood pathologies accompanied by myeloproliferation, erythremia is the most common chronic ailment. Of the one hundred thousand people, the true polycythemia is affected by twenty-nine people.
Disease eritremia manifests slowly, for a while a person does not even suspect that he is sick. Over time, the disease makes itself felt, the clinic is dominated by the phenomena of plethora, as well as related complications. So, on the skin, and especially on the neck, swollen large veins become noticeable. Skin polycythemia has a cherry color, especially pronounced such a bright color in open areas (face, hands). Lips and tongue take a red-blue hue, hyperemic and conjunctiva (eyes are as if filled with blood).
Another characteristic sign of erythremia is a symptom of Cooperman, in which the soft palate differs in color, and the hard palate remains the same. Such colors of the skin and mucous are obtained because the small vessels on the surface are filled with blood, its movement becomes slow. For this reason, almost all of the hemoglobin passes into the reconstituted form.
The second important sign is the itchy skin . It happens to almost half of people who suffer from erythremia. Especially intense, this itch becomes after bathing in warm and hot water. The cause is released serotonin, prostaglandins and histamine. Often occurs erythromelalgia. It will be marked by very severe painful burning at the fingertips. Peculiar pain accompanied by reddening and the formation of cyanotic spots. The cause of burning is a large number of platelets, which causes microthrombi.
Erythremia true is often accompanied by growth in the size of the spleen. The increase in this organ can be of any degree. The liver can suffer also. Hepatomegaly in this case will be caused by high blood filling, direct involvement of the liver in the processes of myeloproliferation.
In some cases of erythremia, ulcers of the duodenum and stomach appear. Ulcer of the duodenum in these patients is more common. This is due to the fact that blood clots in mucosal vessels lead to worsening of trophism, which reduces the ability of the body to restrain the growth of Helicobacter pylori.
Another dangerous symptom is the development of blood clots in blood vessels. Earlier thrombi became the main cause of death in erythremia. Clots form in patients with this disease due to excessively high viscosity, changes in the wall of blood vessels. This causes a blood circulation defect in the veins of the brain, legs, spleen vessels, and also in the coronary vessels. Despite the high ability of the body to thrombosis, erythremia can be accompanied by bleeding. Quite often, bleeding occurs from the esophagus veins, gums.
Erythemia can also be accompanied by arthritic pains of a gouty character. The reason is an increase in the level of uric acid. Based on the monitoring results, every fifth patient with erythremia notes this symptom. True polycythemia is often accompanied by obliterating endarteritis , so patients complain of pain in their legs. The pain will be caused by the aforementioned erythromelalgia. Hyperplasia of CM is indicated by pain under pressure or effleurage of flat bones.
Subjective symptoms that a patient with erythremia can indicate: fatigue, noise, ringing in the ears, "flies" before the eyes, headaches, poor vision, dizziness , dyspnea. Because of the viscosity of the blood, the pressure in patients is consistently elevated. With a long course of the disease, cardiosclerosis , heart failure may appear.
Erythremia passes through 3 stages. With initial erythrocytosis moderate, in CMC - panmyelosis. There are no vascular, organ complications yet. Slightly enlarged spleen. This stage can last five or more years. In the proliferative phase, the plethora and hepatosplenomegaly are pronounced due to myeloid metaplasia. Patients begin to deplete. In the blood, the picture is different. There can be exclusively erythrocytosis or thrombocytosis with erythrocytosis, or panmelosis. The variant of neutrophilia and a shift to the left also can not be ruled out. In serum, uric acid is significantly increased. Eritrea in the phase of exhaustion (the third stage) is characterized by a large liver, a spleen, in which myelodysplasia is found. In the blood pancytopenia grows, in CMC - myelofibrosis.
Erythemia is accompanied by a decrease in weight, a symptom of "sock and gloves" (legs and hands change color intensively). Erythremia is also accompanied by high blood pressure, increased predisposition to bronchitis , respiratory diseases. During trepanobiopsy, a hyperplastic process (of a productive nature) is diagnosed.
Erythremia blood test
The laboratory data for erythremia are very different from those of a healthy person. So, the indicator of the number of red blood cells is significantly increased. Hemoglobin blood also grows, it can be 180-220 grams per liter. The color index for this disease, as a rule, is below one, and is 0.7-0.8. The total volume of blood circulating through the body is much more than normal (in one and a half - two and a half times). This is due to the increase in the number of red blood cells. Hematocrit (the ratio of blood elements to plasma) is also rapidly changing due to increased erythrocytes. He can reach the level of sixty-five or more percent. The fact that the regeneration of erythrocytes in erythremia proceeds in an accelerated mode is indicated by a high number of reticulocyte cells. Their percentage can reach fifteen to twenty percent. In the smear, you can find erythroblasts (single), the blood reveals polychromasia of red blood cells.
The number of white blood cells is also increasing, usually one and a half to two times. In some cases, leukocytosis may be even more pronounced. An increase is due to the sharp growth of neutrophils, which reach seventy to eighty percent, and sometimes even more. Sometimes there is a shift in myelocyte character, more often a stab. The fraction of eosinophils also grows, sometimes together with basophils. The number of platelets can grow up to 400-600 * 10 9 liters. Sometimes platelets can reach large markers. Seriously increased and the viscosity of the blood. The rate of erythrocyte sedimentation does not exceed two millimeters per hour. The amount of uric acid also rises, sometimes rapidly.
You should know that for a diagnosis, only a blood test will not be enough. The diagnosis of "erythremia" is based on a clinic (complaints), high hemoglobin, a large number of erythrocytes. Together with the analysis of blood in erythremia, the bone marrow is also being performed. In it, the proliferation of CM elements can be found, in most cases this is due to the progenitor cells of red blood cells. At the same time, the ability to mature in cells in the bone marrow remains at the same level. This disease must be differentiated with various secondary erythrocytosis, which appear due to reactive stimulation of erythropoiesis.
Erythemia occurs in the form of a long, chronic process. The risk to life is reduced to a high risk of bleeding and the formation of blood clots.
At the very beginning of the development of the erythremia disease, measures aimed at general strengthening are shown: a normal regimen, both work and rest, walks, minimizing sunbathing, physiotherapy. Diet with erythremia - milk and vegetable. Animal proteins should be limited, but not excluded. Do not eat foods that contain a lot of ascorbic acid, iron.
The main goal of the therapy of erythremia is the normalization of hemoglobin (up to one hundred and forty-one hundred and fifty), and the hematocrit - up to forty-five to forty-six percent. It is also necessary to minimize the complications that were caused by transformations in the peripheral blood with erythremia: pain in the extremities, lack of iron, problems with blood circulation in the brain, and organs.
In order to normalize the hematocrit with hemoglobin, the procedure of bloodletting is still applied. The volume of bloodletting with erythremia is five hundred milliliters at a time. Bleeding is done once in two days or every four to five days until the above indicators are normalized. This method is acceptable within the framework of urgent measures, since it stimulates the bone marrow, especially the function of thrombopoiesis. With the same purpose, erythrocytapheresis can also be used. With this manipulation, only the erythrocyte mass is recovered from the blood stream, returning the plasma. Do this often, too, every other day, using a special filtering device.
If erythremia is accompanied by intense itching, an increase in the leukocyte fraction, as well as platelets, a large spleen, diseases of internal organs (JAJ or DPC, IHD, problems in the cerebral circulation), vascular complications (thrombosis of the arteries, veins), then cytostatics are used. These drugs are used to suppress the multiplication of different cells. They include Imiphos, Myelosan, as well as radioactive phosphorus (P32).
The most effective is phosphorus, as it accumulates in high doses in the bones, thereby inhibiting the medullar function, affecting erythropoiesis. P32 is administered orally three to four times at 2 mC. The interval between taking two doses is from five days to a week. The course requires six to eight mC. If the treatment is successful, the patient will be in remission for two to three years. This remission is both clinical and hematological. If the effect is insufficient, the course is repeated a few months later (usually three to four). From the reception of these drugs, a cytopenic syndrome may appear, which is more likely to develop even into osteomyelophybrosis, chronic myelogenous leukemia . In order to avoid such unpleasant outcomes, as well as with metaplasia of the liver, the spleen, it is necessary to keep under control the total dose of the drug. The doctor must ensure that the patient does not take more than thirty mC.
Imiphos in erythremia is expressed by inhibiting the reproduction of erythrocytes. The course requires five hundred to six hundred milligrams of Imiphos. It is administered fifty milligrams a day. The duration of remission is from six months to one and a half years. It should be remembered that this agent has a detrimental effect on the myeloid tissue (contains myelotoxins), and this causes hemolysis of the erythrocytes. That is why this drug with erythremia should be used especially carefully if the spleen and platelet fractions are not involved in the process, the leukocytes differ little from the norm.
Myelosan is not a means of choice in erythremia, but it is occasionally prescribed. This drug is not used if the leuko-, platelets are within the limits of normal or reduced. As an additional means of using anticoagulant drugs (in the presence of thrombosis). Such treatment is performed exclusively under the strict control of the prothrombin index (it should be at least sixty percent).
From anticoagulants of indirect influence to patients with erythremia give Phenilin. It is taken daily for three hundred milligrams. Use and disaggregants of platelets (acetyl acid for five hundred milligrams daily). After taking Aspirin, a physiological solution is injected into the vein. Such a sequence is necessary in order to eliminate the plethora.
If erythremia is treated under the conditions of the ward regime, it is advisable to use Myelobromol. Assign daily to two hundred and fifty milligrams. When the leukocytes begin to fall, the drug is given after a day. Completely abolished, when the leukocytes are reduced to 5 * 10 2 liters. Chlorbutin is prescribed eight to ten milligrams per day. The duration of therapy with this drug with erythremia is about six weeks. After a while, treatment with Chlorbutin is repeated. Before the onset of remission, patients should take Cyclophosphamide daily for one hundred milligrams.
If the hemolytic anemia of autoimmune genesis begins with erythremia, glucocorticosteroids are used. Preference is given to Prednisolone. He is prescribed thirty-sixty milligrams a day. If such treatment has not given any special results, the patient is recommended splenectomy (surgical manipulation to remove the spleen). If erythremia has passed into leukemia with acute course, it is treated according to the appropriate therapy regimen.
Since almost all iron is bound to hemoglobin, other organs do not receive it. That the patient did not have a deficiency of this element, with erythremia, iron preparations are introduced into the scheme. These include Hemofer, Ferrum Lek, Sorbifer, Totem.
Hemofer - a drop that appoints fifty-five drops (two milliliters) twice a day. When the level of iron is normalized, in order to prevent the dose is reduced by half. The minimum duration of treatment for erythremia with this remedy is eight weeks. Improvement of the picture will be noticeable in two to three months from the moment of the beginning of therapy by Hemofere. The drug can sometimes have an effect on the digestive tract, causing poor appetite, nausea with vomiting, a feeling of over-filling epigastrium, constipation or, conversely, diarrhea .
Ferrum Lek can only be injected into the muscle, intravenously not. Before starting treatment, give a test dose of this drug (half or quarter of the ampoule). If no undesirable effects are detected within a quarter of an hour, the remaining amount of the drug is administered. The dose of the drug is prescribed individually, taking into account the indices of iron deficiency. The usual dose for erythremia is one or two ampoules of the drug per day (one hundred two hundred milligrams). The contents of two ampoules are administered only if the hemoglobin is too high. Ferrum Lek must be injected deep, into the left, right buttocks alternately. To reduce the pain when administered, the drug is stuck in the outer quadrant with a needle, the length of which is at least five centimeters. After the skin is treated with disinfectant, before inserting the needle, it must be moved a couple of centimeters down. This is necessary to prevent the backflow of Ferrum Leka, which can lead to the dyeing of the skin. Immediately after the injection, the skin is released, and the injection site is tightly pressed with the fingers with cotton wool, keeping it for at least a minute. It is necessary to pay attention to the ampoule before taking an injection: the drug should look homogeneous, without sediment. Enter it immediately after opening the ampoule.
Sorbifer is taken internally. Drink one tablet twice a day before breakfast and dinner for thirty minutes. If the drug caused side effects (an unpleasant aftertaste in the mouth, nausea), you need to switch to a single dose (one tablet). Therapy Sorbiferum with erythremia is carried out under the control of iron in the channel. After bringing the level of iron back to normal, treatment should continue for another two months. If the case is severe, the duration of treatment can be increased to four to six months.
One ampoule of Totem is added to water or a drink that does not contain ethanol. Drink better on an empty stomach. Assign a hundred - two hundred milligrams per day. The duration of therapy with erythremia is three to six months. Totem should not be taken if erythremia is accompanied by JAJ or JAHPK, hemolytic form of anemia, aplastic and sideroachrastic anemia, hemosiderosis, hemochromatosis.
Erythema can also be accompanied by urate diathesis. It is caused by rapid destruction of erythrocytes, accompanied by the ingestion of various metabolic products into the blood. To bring urates in norm with erythremia is possible by means of such medical preparation, as Allopurinol (Milurit). The daily dosage of the drug is variable, depending on the severity of the flow, the amount of uric acid in the body. Usually the amount of the drug ranges from one hundred milligrams to one gram. One gram is the maximum dose given in exceptional cases. Often a hundred - two hundred milligrams for the diagnosis of "erythremia" is quite enough. It is important to know that Milurit (or Allopurinol) can not be taken if erythremia is accompanied by renal insufficiency or an allergy to any constituents of this medication. Treatment should be long, breaks between the methods of the remedy for more than two days are unacceptable. When treating erythremia with this remedy, you need to drink plenty of water in order to ensure a diuresis of at least two liters per day. This drug is not recommended for use during antitumor therapy, since Allopurinol makes these drugs more toxic. If it is impossible to avoid simultaneous reception, the dose of cytostatic is reduced by half. When Milurita is used, the effect of indirect anticoagulants (including undesirable effects) is increased. Also, this drug should not be consumed simultaneously with iron preparations, since this can contribute to the accumulation of the element in the liver.