Erythremia
Description:
Erythremia - a chronic leukosis with defeat at the level of a cell - predecessors of a myelopoiesis with the unlimited growth of this cell, characteristic of the tumor, which kept ability to be differentiated on 4 sprouts it is preferential on red. At certain stages of a disease, and sometimes and from the very beginning, the myeloid metaplasia in a spleen joins growth of cells in marrow.
Erythremia symptoms:
The disease begins gradually. Reddening of integuments, weakness, weight in the head, increase in a spleen, an arterial hypertension, and at a half of patients - a painful skin itch after washing, washing, swimming accrues. Sometimes necroses of fingers, fibrinferments of larger arteries of the lower and upper extremities, thrombophlebitis, a trombotichesky stroke, a myocardial infarction or a lung and especially acute thermalgias in finger-tips eliminated by acetylsalicylic acid for 1-3 days become the first displays of a disease. At many patients long before establishment of the diagnosis bleedings after an exodontia, a skin itch after a bathtub and "good" indicators of red blood to which doctors did not attach due significance were observed.
In the I stage which duration makes 5 years and more moderate increase in the circulating blood is observed, the spleen is not probed. In blood at this stage moderate formation of erythrocytes prevails. In marrow increase in all sprouts of a hemopoiesis. Vascular and visceral complications are possible at this time, but are not frequent.
Allocation of an initial (I) stage of an erythremia is conditional. In essence, it is a stage with oligosymptomatic manifestations, more inherent to elderly patients. The spleen usually is not probed, but its research quite often reveals small increase. Trombotichesky complications are possible also in this stage of a disease.
IIA the process stage - eritremichesky - is developed, for it uncharacteristically myeloid transformation of a spleen. Duration of this stage makes 10-15 years and more. The volume of the circulating blood is increased, the spleen is increased, and increase in a liver is a little earlier possible. Fibrinferments of arterial and venous vessels, hemorrhagic complications at this stage are observed more often. Blood test indicates a "pure" erythrocytemia or an erythrocytemia and a thrombocytosis or a panmyelosis and a neutrocytosis with band shift, increase in number of basophiles. In marrow the total trekhrostkovy hyperplasia with expressed megakariotsitozy is observed, myelofibroses are possible reticulin and focal collagenic.
The developed process, but with a myeloid metaplasia of a spleen also belongs to stage IIB eritremicheskiya. Increase in volume of blood can be expressed to a greater or lesser extent, increase in a liver and spleen is observed. In blood in this stage increase in erythrocytes is noted, it is higher than 15 P 103 of thrombocytes with a leukocytosis in 1 мкл and shift of a leukocytic formula to myelocytes, single eritrokariotsita. In marrow, as well as in IIA of a stage, increase in a granulotsitarny sprout can prevail, the reticulin and focal collagenic myelofibrosis is possible.
In a clinical picture quite often leading are allergic complications and uratovy diathesis.
In this stage exhaustion of the patient, the becoming aggravated trombotichesky complications and bleeding can be observed.
The III stage of an erythremia call anemic. In marrow the myelofibrosis can be expressed, the myelopoiesis in one cases is kept, and in others is lowered. In the increased spleen and a liver myeloid transformation is observed. The acute leukosis, a myelosis, a hypoplastic condition of a blood formation and difficult classified hematologic changes can be an erythremia outcome in this stage.
The arterial hypertension arising at an erythremia in 35-50% of cases is caused by increase in peripheric resistance in response to the increased viscosity of blood, development of uratovy diathesis, chronic pyelonephritis, disturbances of blood circulation in a parenchyma of kidneys, thrombosis and a sclerosis of renal arteries.
The skin itch, specific to erythremia, connected with washing is observed at 50-55% of patients. At many patients it becomes the main complaint, arises not only from contact with water, but also is spontaneous, affects working capacity.
Frequent complications of the developed stage of a disease are microcirculator frustration with clinic of an eritromelalgiya, passing disturbances of cerebral and coronary circulation and hemorrhagic hypostases of shins, and also fibrinferments of venous and arterial vessels and bleeding. At this stage there can already be disturbances of a hemostasis which look quite often as the latent thrombogenic danger revealed only laboratory and which does not have clinical manifestations. At the same time disturbances of a hemostasis can be and more expressed, to lead to local intravascular coagulation as microthromboses or to the disseminated intravascular coagulation - to the IDCS.
The mechanism of development of trombotichesky complications of an erythremia consists in increase in mass of the circulating erythrocytes, delay of a blood flow and increase in its viscosity. Their development is promoted by a thrombocytosis and high-quality disturbances of thrombocytes. In a blood plasma the circulating units of thrombocytes quite often are defined that happens the investigation not only their quantitative increase, but also disturbance of functional properties of thrombocytes.
Hemorrhagic complications of an erythremia are completely liquidated at patients, treated by bloodlettings when the hematocrit indicator is normalized.
With development of an erythremia the deficit of iron eliminating a plethora is quite often observed. Clinical manifestations of deficit of iron - weakness, a language inflammation, decrease in resilience to infections, thinning of nails - are more often observed at senior citizens.
Development of an anemic stage is preceded by certain dynamics of kliniko-hemorrhagic data, in particular increase in a spleen, gradual reduction of a plethora, emergence of a leykoeritroblastichesky picture of peripheral blood. In marrow the myelofibrosis which can be accompanied change of type, cellular growth, increase of pathology of vessels of marrow and inefficiency of a hemopoiesis - an erythremia outcome gradually develops in a secondary myelofibrosis.
There are also other forms and options of a course of a disease at which increase in a spleen due to myeloid transformation from the very beginning comes to light. Exacerbations of a disease after treatment of cytostatics proceed preferential with a plethora and increase in a spleen. These are always pantsitozny forms of a disease with a leykoeritroblastichesky picture of blood, heavier, than a usual erythremia.
From an erythremia they differ in the early and expressed extra marrowy distribution, a bigger trekhrostkovy orientation of growth and a reticulin myelofibrosis, and from an idiopathic myelofibrosis - existence of a plethora and duration of a miyeloproliferation, lack of a tendency to bystry end of a reticulin myelofibrosis.
At the same time the anemia developing at an erythremia can have various mechanism of development, is not always connected with a progression of process and in many cases with success is treated.
Anemia can be iron deficiency, caused by bleedings and bloodlettings; gemodilyutsionny, the circulating plasma connected with increase in volume owing to increase in a spleen, the hemolitic, caused by increase function of a spleen. At last, anemia at an erythremia can be a consequence of inefficient blood formation. At an erythremia outcome in an acute leukosis or in a hypoplasia of a hemopoiesis the anemia inherent to these processes is observed.
Erythremia outcome frequency in an acute leukosis makes 1% at not treated and 11-15% at treated cytostatics (Chlorbutinum), the acute miyeloblastny leukosis and an erythremic myelosis develops more often. The harbingers of an acute leukosis arising sometimes 2-3 years prior to its diagnosis are noninfectious fever, unmotivated a leukopenia, trombo-or a pancytopenia, sometimes dermatitis.
Posteritremichesky myelofibrosis - result of natural evolution of a disease. It is observed at each patient with an erythremia living up to this period. Distinction of its hematologic manifestations and a current - from high-quality, with hematologic compensation, to malignant, with a bystry anemization, a depression granulo-and a thrombocytopoiesis, sometimes with a low-percentage blastemiya is amazing. In these cases it is probably necessary to assume a tumoral progression of a disease before which manifestations in the form of blast crisis there can pass months and years.
Erythremia reasons:
Specific cytogenetic anomalies at an erythremia are not found.
Quantitative defects of chromosomes, structural aberrations have clonal character and are not found in lymphocytes. At the patients who underwent treatment by cytostatics they meet more often. According to authors, patients with originally
are not predisposed with the found disturbances of chromosomal complement to more malignant course of a disease.
Though morphological, fermental and cytogenetic features of damage of lymphatic system at an erythremia are not available, the functional condition of T lymphocytes is changed: the reduced answer to the known mitogens and increase in their spontaneous activity is found.
In an eritremichesky stage in marrow full disturbance of structure of sprouts with fat replacement is usually observed.
In addition classical option, changes of 3 more types can be observed: increase in erythroidal and megakariotsitarny sprouts, increase in erythroidal and granulotsitarny sprouts; increase in preferential erythroidal sprout. Iron reserves in marrow are considerably reduced. The base of a hemopoiesis is often expanded, fatty marrow can look red, hemopoietic.
The spleen is overflowed with blood, contains sites of heart attacks of various prescription, units of thrombocytes and quite often initial, moderate or considerable symptoms of a myeloid metaplasia with localization in sine. The follicular structure is usually kept.
In a liver along with a plethora the fibrosis centers, connection of hepatic beams, sometimes a myeloid metaplasia with localization in sinusoids are observed. In a gall bladder very dense bile and pigmental stones are often visible.
Frequent find are uratovy stones, pyelonephritis, contracted kidneys, considerable pathology of their vessels.
In an anemic stage of a disease the expressed myeloid transformation of a spleen and liver, and also their increase is observed. Marrow often фиброзирован. At the same time myeloid fabric can be both giperplazirovanny, and reduced, vessels of marrow are sharply increased in quantity and structurally changed. In parenchymatous bodies dystrophic and sclerous changes come to light. Manifestations of a trombotichesky syndrome or hemorrhagic diathesis are frequent.
The functional condition of products of erythrocytes, according to radiological researches, is sharply strengthened: the elimination half-life of the radioactive iron entered into a vein is shortened, its utilization is strengthened by marrow and the circulation is accelerated.
Average life expectancy of thrombocytes is often shortened, there is a negative communication between their survival and size of a spleen.
Treatment of the Erythremia:
Problem of treatment - normalization of amount of hemoglobin to 140-150 g/l (85-90 PIECES) and a hematocrit indicator (46-47%) as at the same time the risk of vascular complications sharply decreases. Bloodlettings are appointed on 500 ml every other day in a hospital and in 2 days at out-patient treatment. Instead of bloodlettings it is better to carry out эритроцитаферез. The number of bloodlettings is defined by achievement of normal indicators of red blood.
At the patients of advanced age either having associated diseases of serdechnoyososudisty system, or badly transferring bloodlettings once delete no more than 350 ml of blood, and intervals between bloodlettings extend a little. For simplification of bloodlettings and prevention of trombotichesky complications the day before and in day of the procedure or during the entire period of bloodlettings, and also 1-2 weeks after the end of treatment it is necessary to appoint dezagregantny therapy - acetylsalicylic acid on 0,5-1 g/days and curantyl on 150-200 mg/days at the same time. In addition just before bloodletting introduction of 400 ml of a reopoliglyukin is recommended.
At contraindications to use of acetylsalicylic acid the doctor appoints curantyl, a papaverine or drugs of niacin. Upon termination of treatment the condition of patients and a pattern of blood are controlled by each 6-8 weeks.
As the indication to purpose of tsitostatik serve an erythremia with a leukocytosis, a thrombocytosis and increase in a spleen, a skin itch, visceral and vascular complications, serious condition of the patient, and also insufficient efficiency of the previous treatment with bloodlettings, need for their frequent repetition, bad portability and a complication with both a stable thrombocytosis, and clinically shown deficit of iron. In the latter case against the background of treatment cytostatics carry out replacement therapy by iron preparations. Advanced age of patients (50 years are more senior), impossibility to organize therapy by bloodlettings expand indications to treatment with cytostatics.
Cytostatic therapy is usually combined with the bloodlettings appointed before normalization of a hematocrit and amount of hemoglobin from the very beginning of cytostatic therapy.
Hematologic control of the course of treatment is carried out weekly, and by the end of treatment - each 5 days.
The maintenance therapy is not recommended by cytostatics because of small efficiency and danger of leykozogenny action. Preferably timely course treatment in the full or reduced volume at tendency to aggravations.
Uratovy diathesis is the indication to purpose of Miluritum (Allopyrinolum) in a daily dose from 0,3 to 1 g. Drug reduces synthesis of uric acid from hypoxanthine which content increases owing to a cellular hyper catabolism. At treatment cytostatics drug is appointed preventively in a daily dose from 200 to 500 mg and more.
The microcirculator frustration and, in particular, eritromelalgiya (attacks of sudden thermalgias preferential in extremities with local reddening and a cutaneous dropsy) caused by preferential aggregation block of an arterial blood-groove on at – the aries of capillaries and small arteries, with success are treated by acetylsalicylic acid on 0,31 g a day. Efficiency of one curantyl at an eritromelalgiya is much lower.
It should be noted the gastrointestinal bleedings which developed in connection with broad use of acetylsalicylic acid, including long and constituting real danger. Long nasal and gingival bleedings are possible.
This complication of treatment is caused as not recognizable digestive tract cankers inherent to an erythremia and proceeding asymptomatically and the initial functional deficiency of thrombocytes aggravated by acetylsalicylic acid.
Acute fibrinferments of vessels - the indication to appointment not only dezagregant of thrombocytes, but also heparin, transfusions of freshly frozen plasma.
At treatment in an anemic phase consider the mechanism of development of anemia, thrombocytopenia and other symptoms. At the anemia caused by deficit of iron or folic acid the corresponding replacement therapy is appointed. Treatment of gemodilyutsionny anemia has to be directed to reduction of a spleen by means of radiation therapy, tsitostatik and Prednisolonum. The anemia caused by insufficient formation of erythrocytes it is preferable to treat androgens or anabolic steroids. Prednisolonum is appointed preferential at suspicion to an autoimmune origin of anemia and thrombocytopenia, and also for the purpose of reduction of a spleen.
