Aplastic anemias — heterogeneous group of diseases of system of blood which basis is made by reduction of products of cells of marrow are more often than three cellular lines (eritrotsito-, leucio-and a thrombocytopoiesis).
It is not enough data on epidemiology of aplastic anemias. In some states of the USA (for example, in California) incidence of aplastic anemias makes about 1:300 000 — 1:500 000. Noticeable dependence of incidence on a floor and an ethnic origin is not revealed. The disease can arise at persons of any age, however is more often at young people (from 20 to 40 years).
Etiology and pathogeny
In 50 — 75% of cases the reason of aplastic anemias remains to the unknown. Their development can be caused by various exogenous factors with obligate action (the ionizing radiation, benzene and its derivatives, antineoplastic himiopreparata, inorganic compounds of arsenic, etc.) or optional myelotoxic action (streptomycin, levomycetinum, anticonvulsant, anti-thyroid, antihistaminic, antimalarial and other means). Chances of aplastic anemias at tuberculosis, pregnancy, hepatitis. From internal causes attach significance to estrogen. The etiology of constitutional aplastic anemia is connected most likely with inheritance of a pathological gene from one of parents on recessive type. Cases of the children who were born from interrelated marriages are described. Most a popular belief in defeat at aplastic anemias of partially determined stem cell and/or its microenvironment, about influence of various populations of lymphocytes on a stem cell. Autoimmune genesis of a disease is proved for part of cases of a partial krasnokletochny aplasia (PKKA).
Classification of aplastic anemias
Distinguish from aplastic anemias constitutional and acquired. Along with a typiform the disease options proceeding in the form of a partial krasnokletochny aplasia or which are followed by periodic development of an intravascular hemolysis as a paroxysmal night haemoglobinuria meet (at the beginning of formation of an illness or at separate stages of process). At children allocate the acquired, family and inborn forms of aplastic anemias.
Approximate formulation of the diagnosis: 1. Aplastic anemia with a pancytopenia (eritro-, trombo-, a leukopenia with a neutropenia and a relative lymphocytosis), a miyelokariotsitopeniya, hemorrhages (bruises on skin, gingival and nasal bleedings). 2. The aplastic anemia with a pancytopenia, a miyelokariotsitopeniya and a hemorrhagic syndrome proceeding periodically with viutrisosudisty hemolysis (as a paroxysmal night haemoglobinuria). 3. A partial krasnokletochny aplasia with the expressed anemia and reduction of an erythrogenesis in marrow.
Patients with aplastic anemias are pale, with the kept or excess hypodermic fatty layer. They complain of the general weakness, fast fatigue, the lowered working capacity. Usually there are hemorrhages in skin and mucous membranes, bleedings from a nose and gums, uterine, gastrointestinal and renal bleedings, hemorrhages in a brain and any other body.
Treat characteristic symptoms of a disease: a pancytopenia (eritro-, trombo-, a leykotsitopeniya with a neutropenia and a relative lymphocytosis); in a miyelogramma — reduction of quantity of myelocariocytes, relative increase of maintenance of lymphocytes, absolute decrease in quantity of neutrophils, erythrogenesis elements, sharp reduction of a megakariotsitopoez; in a trepanobioptata of an ileal bone fatty marrow prevails over active, in the remained sites of a hemopoiesis lymphocytes, eritro-and the normoblasts which are often containing iron granules meet mainly; more than in 90% of cases intracellular hyper hemolysis with destruction of erythrocytes, mainly in a spleen takes place. In various terms the disease sometimes is complicated by the intravascular hemolysis inherent in a paroxysmal night haemoglobinuria. In these cases yellowness of integuments and visible mucous membranes, dark urine, insignificant increase in a liver come to light, it is rare — spleens and development of thromboses of multiple localization. Existence of an intravascular hemolysis, is confirmed by increase of free fraction of bilirubin of blood serum and free hemoglobin of plasma, Ham and Hartman's positive tests.
Among aplastic anemias at children along with the acquired forms, significantly not different from aplastic anemias at adults, allocate inborn and family options.
Fankoni's anemia having family character meets among boys more often. The first symptoms of an illness are usually shown at the age of 6 — 8. Changes of a hemogenesis are combined with a hemorrhagic syndrome and various anomalies of a skeletogeny and internals in the form of reduction of number of bones of a wrist, absence or a hypoplasia of a thumb, a clinodactyly, a nanocephalia, deformation of a thorax, malformations of a respiratory organs, kidneys and urinary tract, inborn heart diseases and other changes. Also endocrine disturbances in the form of a xanthopathy and mucous membranes which expressiveness usually corresponds to weight of a course of process are observed.
Inborn partial aplastic anemia of Joseph — Dayemonda — Blekfena develops at early children's age. Perhaps, it is connected with the autoimmune process arising during pre-natal development or with inborn metabolic defect. Anemia of various degree is combined with a deep eritro-and a normoblastopeniya against a miyelokariotsitopeniya. Leykopeez and a thrombopoiesis are kept, bleeding is not observed.
The same changes from a hemopoiesis are characteristic of family aplastic anemia of Estrenum — Damesheka that at aplastic anemias of adults. Unlike aplastic anemia of Fankoni at this form anomalies of a skeletogeny and internals are not observed.
Verification of the diagnosis
At detection of the kliniko-hematologic data characteristic of aplastic anemia, establishment of the diagnosis does not cause difficulties. If the disease proceeds with a syndrome of an intravascular hemolysis, the diagnosis is established on the basis of clinical symptomatology and results of laboratory researches.
If aplastic anemia precedes developing of an acute leukosis, the diagnosis is established by means of studying of a miyelogramma and especially histologic picture of marrow. At malignant new growths (is more often — a digestive tract) owing to toxic influence of a tumor and/or innidiation the hemopoiesis depression can develop in marrow. It meets at an osteosclerosis, an osteomyelofibrosis (the sizes of a liver, spleen, etc. are considered given to a X-ray analysis of bones of a skeleton, a trepanobiopsiya).
Treatment of aplastic anemias
For stopping of anemia use eritrotsitny weight, is more often — the washed or defrozen erythrocytes (the purpose of reduction of an isosensitization of the patient). Hemorrhages serve as the indication to use of transfusions of trombotsitny weight. In the absence of haemo static effect use 2 — 5 plasma exchanges with removal on 1 — 1,5 l of plasma with substitution of volume m quantity svezhezamorozhen are adequate ache donor plasma, albumine or a reopoliglyukin. It is reasonable to carry out a plasma exchange with an interval of 8 — 10 days against continuation of reception of Prednisolonum in the doses promoting reduction of hemorrhagic manifestations. Apply the means exerting beneficial effect on a vascular wall (Dicynonum, serotonin, Rutinum, ascorbic acid), aminocapronic acid at the corresponding disturbances of a hemostasis, androgens (retabolil, anapolon, etc.) it is frequent in combination with glucocorticoids; desferal for the purpose of reduction of the phenomena of a hyper siderosis. In the absence of noticeable effect of such therapy recommend a splenectomy which is made at rather favorable indicators of a gemogramma, a condition of a marrowy hemopoiesis and lack of bleeding. There are uses of endovascular occlusion of a splenic artery given about expediency. Clinical experience by efficiency of use of anti-lymphocytic globulin and cyclosporine A is saved up.
Transplantation of marrow at heavy aplastic anemia is applied no later than 3 months from the moment of establishment of the diagnosis, it is preferable — the patient with the minimum quantity of the made hemotransfusions. The age of patients matters also: the best results are received at persons 30 years are not more senior. At this disease pretransplant preparation includes Cyclophosphanum intravenously in a total dose of 200 mg/kg within 4 days or Cyclophosphanum combination to fractional irradiation of an adenoid tissue in the general dose of 7,5 — 8 Gr for 3 — 4 sessions. Remissions after a miyelotransplantation are noted at 80 — 90% of patients with heavy aplastic anemia.