Polycythemia
Description:
Osler's disease, hyperglobulia, erythremia, erythremia - a chronic disease, which reason - defeat of a cell - predecessors of a myelopoiesis, shown unlimited erythroidal proliferation and the kept ability of a differentiation on 4 sprouts of a hemopoiesis. On structure and annual average indicators of incidence the polycythemia takes the 4th place after a myelosis. A polycythemia - a disease of preferential elderly and old people (middle age - 60 years). Cases and at young and children's age are frequent. At young people the disease proceeds more adversely.
Polycythemia symptoms:
The polycythemia is characterized by long and rather high-quality current.
In a clinical current distinguish several stages:
* initial, or oligosymptomatic, a stage, usually lasting 5 years, with the minimum clinical manifestations;
* IIA stage - the eritremichesky developed stage, without myeloid metaplasia of a spleen, its duration can reaches 10-20 years;
* IIB stage - the eritremichesky developed stage, with a myeloid metaplasia of a spleen;
* a stage of III - a stage of a posteritremichesky myeloid metaplasia (an anemic stage) with a myelofibrosis or without it; the outcome in an acute leukosis, a myelosis is possible.
However, considering a usual onset of the illness at elderly and old people, not at all patients it passes all three stages.
In the anamnesis many patients long before the moment of diagnosis have instructions on bleedings after extraction of tooth, the skin itch connected with hydrotherapeutic procedures, the "good", a little overestimated indicators of red blood, a duodenum ulcer. Increase in mass of the circulating erythrocytes leads to increase in viscosity of blood, staza in a microcirculator bed, to increase in peripheric vascular resistance therefore face skin, ears, a tip of a nose, distal departments of fingers and visible mucous have red and cyanochroic coloring of various degree. Are explained by the increased viscosity high frequency vascular, preferential cerebral, complaints: a headache, dizziness, sleeplessness, feeling of weight in the head, a vision disorder, a sonitus. Epileptiform attacks, a depression, paralyzes are possible. Patients complain of the progressing decrease in memory. In an initial stage of a disease the arterial hypertension is found in 35-40% of patients. The cellular hyper catabolism and partially inefficient erythrogenesis become the reason of the increased endogenous synthesis of uric acid and disturbance of exchange of urates. Clinical displays of uraty (urate) diathesis - the renal colic, gout complicating a current of IIB and the III stage. Carry to visceral complications stomach ulcer and a duodenum, frequency makes them, given different authors, from 10 to 17%.
Vascular complications constitute the greatest danger to patients with a polycythemia. Unique feature of this disease is simultaneous tendency as to thrombosis, and bleeding. Microcirculator frustration as a result of a thrombophilia are shown by an eritromelalgiya - the sharp reddening and hypostasis of distal departments of fingers of hands and legs which is followed by a thermalgia. The persistent eritromelalgiya can be a harbinger of thrombosis of larger vessel with development of a necrosis of fingers of hands, feet, shins. Thrombosis of coronary vessels is noted at 7-10% of patients. Development of thrombosis is promoted by a number of factors: the age is more senior than 60 years, fibrinferments of vessels in the anamnesis, an arterial hypertension, atherosclerosis of any localization, an eksfuziya of blood or the plateletpheresis which are carried out without purpose of anticoagulating or dezagregantny therapy. Trombotichesky complications, in particular myocardial infarction, ischemic stroke and thromboembolism of a pulmonary artery, are the most frequent cause of death of these patients.
The hemorrhagic syndrome is shown by the hematostaxic of gums, nasal bleedings, ecchymomas characteristic of disturbances of a platelet and vascular link of a hemostasis. The pathogeny of microcirculator bleeding depends preferential on decrease in aggregation defective, a neoplastic origin of thrombocytes.
The spleen increases in IIA of a stage, as the reason for that serves strengthening of deposition and sequestration of uniform elements of blood. In IIB stage the splenomegaly is caused by the progressing myeloid metaplasia. It is accompanied the left shift in a leukocytic formula, эритрокариоцитоз. Increase in a liver often accompanies a splenomegaly. Liver fibrosis is characteristic of both stages. The current of a posteritremichesky stage is variable. At a part of patients it is quite good-quality, the spleen and a liver increase slowly, indicators of red blood long enough remain within norm. At the same time also bystry progressing of a splenomegaly, increase of anemia, growth of a leukocytosis and development of blast transformation is possible. The acute leukosis can develop both in an eritremichesky stage, and in stages of a posteritremichesky myeloid metaplasia.
Polycythemia reasons:
The hyperglobulia as one of manifestations of pathological process is more often secondary though in a number of regions cases of the family hyperglobulia (a family polycythemia, primary hyperglobulia) inherited on autosomal recessively type are observed. It meets in various geographical areas, the centers of a disease are for the first time revealed among residents of Chuvashia.
Treat the main reasons for a secondary hyperglobulia a fabric hypoxia, both inborn, and acquired, and change of content of endogenous erythropoetin.
Reasons of a secondary hyperglobulia:
A.Vrozhdenny:
1.vysokoye affinity of hemoglobin to oxygen;
2.nizky level is 2,3 diphosphoglycerates;
3.avtonomnaya products of erythropoetin.
B. Acquired:
1.arterialnaya anoxemia of the physiological and pathological nature:
"blue" heart diseases;
chronic pulmonary diseases;
smoking;
adaptation to highlands conditions.
2.zabolevaniya kidneys:
tumors;
hydronephrosis;
cystous defeat;
diffusion diseases of a renal parenchyma;
stenosis of renal arteries.
3.opukholi:
cerebellum hemangioblastoma;
carcinoma of bronchial tubes.
4.endokrinnye diseases:
tumors of adrenal glands.
5.zabolevaniya liver:
hepatitis;
cirrhosis;
Badda-Kiari's syndrome;
gepatoma;
tuberculosis.
Treatment of the Polycythemia:
Acute management. At a polycythemia the main danger are vascular complications. Generally it is gastrointestinal bleedings, preceding infarction stenocardia, a repeated vascular embolism of lungs, arterial and repeated venous thrombosis, i.e. emergency treatment at a polycythemia is generally directed to stopping and further prevention of trombotichesky and hemorrhagic complications.
Planned therapy. Modern therapy of an erythremia consists in use of eksfuziya of blood, cytostatic drugs, use of radioactive phosphorus, and - interferon.
The bloodlettings giving bystry clinical effect can be an independent method of treatment or supplement cytostatic therapy. In the initial stage proceeding with increase in maintenance of erythrocytes apply 2-3 bloodlettings on 500 ml each 3-5 days with the subsequent introduction of adequate quantities of a reopoliglyukin or normal saline solution. At patients with cardiovascular diseases for 1 procedure delete no more than 350 ml of blood, eksfuzy no more than 1 once a week. Bloodlettings do not control quantity of leukocytes and thrombocytes, sometimes being the reason of a reactive thrombocytosis. Usually skin itch, an eritromelalgiya, stomach ulcer and a duodenum, urate diathesis are not eliminated with bloodletting. They can be replaced eritrotsitaferezy with compensation of volume of remote erythrocytes normal saline solution and reopoliglyukiny. The procedure is well postponed by patients and causes normalization of indicators of red blood for a period of 8 up to 12 months.
Cytostatic therapy is directed to suppression of the increased proliferative activity of marrow, its efficiency should be estimated in 3 months after the end of treatment though decrease in quantity of leukocytes and thrombocytes occurs much earlier.
The indication to cytostatic therapy is the erythremia proceeding with a leukocytosis, a thrombocytosis and a splenomegaly, a skin itch, visceral and vascular complications; insufficient effect of the previous bloodlettings, their bad portability.
Contraindications to cytostatic therapy - children's and youthful age of patients, a refrakternost to treatment at the previous stages, also excessively active cytostatic therapy because of danger of a depression of a hemopoiesis is contraindicated.
For treatment of an erythremia the following drugs are used:
* alkylating agents - Myelosanum, алкеран, Cyclophosphanum.
* the hydroxurea which is choice drug in a dose of 40-50 mg/kg/days. After decrease in quantity of leukocytes and thrombocytes the daily dose is reduced to 15 mg/kg by 2-4 weeks, in the subsequent the maintenance dose of 500 mg/days is appointed.
The new direction in therapy of a polycythemia - the use of drugs of interferon oriented to reduction of a miyeloproliferation, quantity of thrombocytes and vascular complications. Time of approach of therapeutic effect - 3-8 months. Normalization of all indicators of blood is estimated as optimum effect, decrease in need for eksfuziya of erythrocytes by 50% - as incomplete. During achievement of effect purpose of 9 million PIECES/days 3 times a week, with transition to the maintenance dose selected individually is recommended. Treatment is usually well transferred and is expected many years. One of undoubted advantages of drug consists in lack of leykozogenny action.
For improvement of quality of life by the patient carry out symptomatic therapy:
* urate diathesis (with clinical manifestations of an urolithiasis, gout) demands constant reception of Allopyrinolum (Miluritum) in a daily dose from 200 mg to 1 g;
* the eritromelalgiya is the indication to purpose of 500 mg of aspirin or 250 mg of a metindol; at a heavy eritromelalgiya heparin is shown in addition;
* at fibrinferment of vessels dezagregant appoint, in case of hypercoagulation according to a koagulogramma it is necessary to appoint heparin in a single dose of 5000 PIECES 2-3 times a day. The dose of heparin is defined by control of coagulant system. In prevention of trombofilichesky complications acetylsalicylic acid is most effective, however its use threatens with hemorrhagic dozozavisimy complications. For a basic preventive dose of aspirin 40 mg of drug a day are accepted;
* the skin itch is a little facilitated by antihistaminic drugs; considerable, but slower (not earlier than 2 months) the effect renders interferon.
