Erythremia - a malignant pathology of blood, accompanied by intense mieloproliferatsiey, leading to the appearance in the bloodstream of a large number of red blood cells and some other cells.Eritremii also called polycythemia. other words a chronic leukemia.
blood cells are produced in excess of have a normal form, structure.Due to the fact that their number increases, the viscosity increases significantly slows blood flow, blood clots begin to develop.All this causes a problem with the blood supply, leading to hypoxia, which increases with time.The first time the disease in the late nineteenth century began Vakeza, and in the first five years of the twentieth century, Osler told about the mechanism of the appearance of this pathology of blood.He erythremia and identified as a separate nosology.
Despite what is known about eritremii almost half a century, it is still poorly understood, reliable cause of the unknown.
Erythremia ICD (International Classifi
believed that there is a family history of the disease.So if eritremii ill close relatives, a chance to get this disease in the future increases.There are some patterns of occurrence of this disease.Ill eritremii mostly people aged (sixty - eighty years), but still there are a few cases when it occurs in children and young people.In young patients erythremia takes very seriously.Men and a half times more likely to suffer illness, but in rare cases of disease among young people is dominated by the female sex.
Of all the pathologies of the blood, accompanied mieloproliferatsiey, erythremia - the most common chronic illness.Out of a hundred thousand people, polycythemia vera suffer twenty-nine people.
erythremia disease manifests slowly, while the man does not even know he was sick.Over time, the disease makes itself felt in the clinic dominated by the phenomenon of plethora, and related complications.So, on the skin, especially around the neck, become swollen conspicuous large vein.The skin has polycythemia cherry color, especially bright colors such pronounced in open areas (face, hands).The lips and tongue are taking red-blue tint, and hyperemic conjunctiva (eye, as it were bloodshot).
Another characteristic feature eritremii - a symptom Cooperman, in which the soft palate is different in color and firm remains the same.Such color skin and mucous membranes prepared from the fact that the small vessels which are on the surface, overflow blood, its movement becomes slow.For this reason, almost all hemoglobin passes into the reduced form.
The second important feature - the itching.It is almost half of the people suffering from eritremii.Especially intense itching that becomes after a swim in the warm and hot water.The reason for this are the released serotonin, prostaglandins, and histamine.Often found rodonalgia.It is marked by a very strong painful burning sensation in the fingertips.A kind of pain is accompanied by redness and cyanotic spots.The cause of burning - a large number of platelets, which is why microthrombuses formed.
Erythremia true often accompanied by an increase in size of the spleen.Increasing the body may be of any degree.May suffer liver.Hepatomegaly in this case is caused by increased blood filling, direct participation in the processes of liver mieloproliferatsii.
eritremii In some cases, noted the appearance of ulcers duodenum, stomach.Duodenal Ulcer in such patients is often.It is connected by blood clots in the vessels of the mucous trophism entail deterioration that reduces the body's ability to inhibit the growth of Helicobacter pylori.
Another dangerous sign - the development of blood clots in the vessels.Previously, blood clots are a major cause of death in eritremii.Blood clots formed in patients with this disease because of the excessively high viscosity, changes in the vascular wall.This defect causes blood circulation in the veins of the brain, legs, blood vessels of the spleen as well as in coronary vessels.Despite the higher the body's ability to thrombosis, erythremia may be accompanied by bleeding.Quite often there are bleeding from esophageal veins, gums.
Erythremia may be accompanied by joint pain and gouty nature.The reason for that - an increase uric acid levels.The monitoring of this symptom notes every fifth patient eritremii people.Polycythemia vera is very often accompanied by occlusive disease, so patients complain of pain in the legs.The pain and cause the aforementioned rodonalgia.On the QM hyperplasia indicates pressure or pain when effleurage flat bones.
subjective symptoms, which may indicate the patient eritremii: fatigue, noise, ringing in the ears, "flies" before the eyes, headaches, poor vision, dizziness, shortness of breath.Due to the viscosity of the blood pressure in patients steadily increased.With long-term course of the disease may appear cardiosclerosis, heart failure.
Erythremia passes 3 stages.With an initial mild polycythemia in CMC - panmieloz.Vascular, organ complications yet.Slightly enlarged spleen.This stage can last five years or more.In the proliferative phase, and the plethora hepatosplenomegaly expressed clearly because myeloid metaplasia.Patients begin to dwindle.The blood picture is different.Maybe only erythrocytosis or thrombocytosis with erythrocytosis or panmieloz.Option neutrophilia and shift to the left, too, can not be excluded.Serum uric acid is significantly increased.Erythremia depletion phase (third stage) is characterized by a large liver, spleen, which are myelodysplasia.In the blood increases pancytopenia in CMC - myelofibrosis.
Erythremia accompanied by weight loss, a symptom of "sock and glove" (arms and legs particularly intensive color change).Erythremia also accompanied by high blood pressure, increased tendency to bronchitis and respiratory diseases.During trepanobiopsy diagnosed hyperplastic process (productive nature).
Laboratory data at eritremii differ from that of a healthy person.Thus, the indicator of the number of red blood cells significantly increased.Hemoglobin increases too, he can reach 180-220 grams per liter.Color indicator for this disease is usually less than unity, and is 0.7 - 0.8.The total volume of blood circulating in the body is much larger than normal (and a half - two and a half times).This is due to the increase in the number of red blood cells.The hematocrit (ratio of blood-plasma) is also changing rapidly by increasing red blood cells.It can reach the mark of sixty-five percent or more.The fact that the regeneration of red blood cells in eritremii proceeds in an accelerated mode, indicates a high number of cells reticulocytes.Their percentage can reach fifteen - twenty percent.The smear can be found erythroblasts (single) in the blood is detected polihromaziya erythrocytes.
number of leukocytes increases also, typically a half to two times.In some cases, leukocytosis may be even more pronounced.The increase contributes to a sharp increase in neutrophils, which reaches seventy - eighty percent, and sometimes more.Sometimes there is a shift myelocytic nature, more often - stab.The fraction of eosinophils is also growing, sometimes together with basophils.The platelet count may grow to 400-600 * 109l.Sometimes platelets can reach and large marks.Seriously, and increased blood viscosity.Erythrocyte sedimentation rate does not exceed two millimeters per hour.The amount of uric acid also increases, sometimes rapidly.
should know that for the diagnosis of a blood test is not enough.Diagnosis "erythremia" put on a clinic-based (complaints), high hemoglobin, a large number of red blood cells.Together with the analysis of blood during eritremii make and bone marrow examination.It can find the elements KM proliferation in most cases this is due to erythrocyte precursor cells.At the same time have the ability to mature cells in the bone marrow remains the same.The disease is to differentiate with different erythrocytosis secondary nature, which are due to reactive stimulation of erythropoiesis.
Erythremia takes the form of long-term, chronic process.Risk of death is reduced to a high risk of bleeding and the formation of blood clots.
At the very beginning of developing the disease eritremii showing events aimed at the general strengthening: normal mode, both labor and leisure, walks, minimize sun exposure, physical therapy measures.Diet for eritremii - lacto-vegetarian.Animal protein should be limited, but not eliminated.You can not eat the foods that contain a lot of ascorbic acid, iron.
main goal of therapy eritremii - bringing back to normal hemoglobin (one hundred forty - one hundred and fifty) and hematocrit - up to forty-five forty-six percent.It is also necessary to minimize the complications that were caused by changes in the peripheral blood eritremii: pain in the limbs, lack of iron, problems with blood circulation in the brain and organs.
In order to normalize the hematocrit, hemoglobin, still using the procedure of bloodletting.The amount of bleeding during eritremii - five hundred milliliters at a time.Rend made every two days or every four to five days to as long as the above figures are not normalized.This method is permissible under the emergency measures, as it stimulates the bone marrow, especially thrombopoiesis function.With the same purpose can be used eritrotsitaferez.With this manipulation of the blood is removed only packed red blood cells, returning the plasma.Do it too often in a day, using a special filtration unit.
If erythremia accompanied by intense itching, increasing leukocyte fractions, as well as platelets, big spleen, diseases of internal organs (GU or KDP, ischemic heart disease, problems in cerebral blood flow), complications from blood vessels (thrombosis of arteries, veins), then use the cytostatics.These drugs are used to suppress the propagation of different cells.These include Imifos, Mielosan and radioactive phosphorus (P32).
phosphorus considered most effective, since it accumulates in high doses in the bone, thereby suppress bone marrow function affects erythropoiesis.P32 is administered orally three or four times to 2 mC.The interval between receiving two doses - from five days to a week.In the course takes six to eight mC.If the treatment was a success, the patient waits in remission for two - three years.Remission this as clinical and hematological.If the effect is insufficient, the course is carried out again after a few months (usually three or four).By taking these drugs may appear tsitopenichesky syndrome, which are most likely to grow even osteomyelofibrosis, chronic myelogenous leukemia.In order to avoid such unpleasant outcomes, as well as metaplasia liver, spleen, must be kept under the control of the total dose.The physician must ensure that the patient has not taken more than thirty mC.
Imifos eritremii when expressed inhibits proliferation of red blood cells.In the course requires five or six hundred milligrams Imifosa.It is administered to fifty milligrams a day.The duration of remission - from six months to one and a half years.Keep in mind that this tool has a detrimental effect on the myeloid tissue (contains mielotoksiny), and this causes hemolysis.That is why this drug should be used during eritremii particularly cautious if the process is not drawn into the spleen and factions of platelets, white blood cells are little different from the norm.
Mielosan is not the drug of choice when eritremii, but rarely prescribed.This drug is not used if the leukocyte, platelets are the results of the study are normal or lowered.As the additional funds used anticoagulant drugs (if thrombosis).This treatment produces only under the strict control of the prothrombin index (it should not be less than sixty percent).
From anticoagulants of indirect exposure to patients with eritremii give Fenilin.It is taken daily for three hundred milligrams.Use and platelet aggregation inhibitors (acetyl acid five hundred milligrams daily).After taking aspirin in the vein injected with saline.This sequence is necessary in order to eliminate the plethora.
If erythremia treated in a ward mode, it is advisable to use Mielobromol.Assign every day for two hundred and fifty milligrams.When white blood cells begin to fall, the drug given in a day.Fully canceled when white blood cells are reduced to 5 * 102l.Hlorbutin appoint eight - ten milligrams orally.Duration of therapy at this facility eritremii - about six weeks.After some time, the treatment is repeated chlorambucil.Until remission patients have to take daily cyclophosphamide hundred milligrams.
If eritremii begins hemolytic anemia of autoimmune origin, used steroids.Preference is given prednisone.He was appointed to the thirty or sixty milligrams per day.If this treatment has not given special results, the patient should splenectomy (surgical procedure to remove the spleen).If erythremia turned into leukemia with acute course, he is being treated for an appropriate treatment regimen.
Since almost all of the iron is associated with hemoglobin, other organs do not get it.To the patient was no shortage of this element, when the scheme is introduced eritremii iron supplements.These include gemofer, Ferrum Lek, Sorbifer, Totem.
gemofer - it drops to appoint fifty-five drops (two milliliters) twice a day.When iron levels to normal in order to prevent reduce the dose by half.Minimum period of treatment at this facility eritremii is eight weeks.Better pictures will be seen in two to three months after initiation of therapy gemofer.The tool can sometimes have effects on the gastrointestinal tract, causing loss of appetite, nausea, vomiting, epigastric sensation of fullness, constipation or, conversely, diarrhea.
Ferrum Lek can be administered only intramuscularly, intravenously impossible.Before treatment should be given a test dose of the drug (half or quarter capsules).If, within a quarter of an hour no adverse effects have been identified, the remaining amount of the drug administered.The dose of the drug administered individually, taking into account indicators of iron deficiency.The usual dose eritremii - one or two vials means a day (one hundred - two hundred milligrams).The contents of the two vials are administered only if the hemoglobin is too high.Ferrum Lek necessarily injected deep in the left, turn right buttock.To reduce the pain when administered, the drug kolyat in the outer quadrant of the needle, the length of which is not less than five centimeters.Once treated skin disinfectants, before introducing it should move the needle two centimeters down.This is to prevent reverse leakage Ferrum Leca, which can lead to staining of the skin.Immediately after injection the skin is released, and the injection site firmly pressed his fingers with cotton wool, so do not hold less than a minute.Note the vial before you make a shot: the drug should look homogenous, without sediment.It should be administered immediately after opening the vial.
Sorbifer taken orally.Drink one tablet twice a day, before breakfast and dinner for thirty minutes.If the drug caused side effects (bad taste in mouth, nausea) should switch to single dose (one tablet).Therapy Sorbifer at eritremii performed under the control of iron in the mainstream.