Acquired dizeritropoeticheskie anemia, related to the violation of the fission process erythrokaryocytes
Etiology and pathogenesis
The concept of "acquired dizeritropoeticheskie anemia" includes a number of clinical entities, common property which is the presence of ineffective erythropoiesis. More-less ineffective erythropoiesis observed in iron deficiency, Thalassemia, aplastic anemia, mielofiʙroze, acute leukemia, However, all these different diseases should not be included in the group of acquired anemia dizeritropoeticheskih.
More appropriate to refer to this group acquired anemia dizeritropoeticheskuyu, related, likely, somatic mutation refractory sideroblastic anemia.
Clinical manifestations and laboratory parameters dizeritropoeticheskih acquired anemias
Refrakternaya sideroblastnaya anemia, most likely, not one nosological form. It is observed when the signs are not very specific to this pathology.
Most often, the disease manifests itself in old age, regardless of gender, It begins slowly. In most cases, first detected mild severe anemia (4,96-5.59 Mmol / l, or 80-90 g / l), marked increase in liver, some patients with palpable spleen. Color index close to unity, However, among the red blood cells can be seen when viewing the stroke two populations - hypochromic and hyperchromic or normochromic. The content of reticulocytes may be normal, sometimes slightly higher, and some patients - a sharp decline. Number of normal leukocytes, sometimes marked leukopenia.
A characteristic feature is pronounced neutrophilic shift. Most often it is limited to a large number of stab neutrophils. Number of individual patients 30-40 %. Often there monocytosis (20-30 %). In the bone marrow has been a sharp irritation of red sprout, leykoeritroidnoe ratio is often lower 1,0. Number oxyphilic normocytes reduced, and increased basophil normocytes. Contents of monocytes in the bone marrow may be normal, despite peripheral monocytosis.
The level of serum iron for refractory sideroblastic anemia may be normal, although most often it is not sharply increased. Thus the amount of iron, excreted in the urine after administration 500 mg Desferal, much higher than normal.
Characteristic of refractory sideroblastic anemia detection is considered in the bone marrow significant number of sideroblasts, t. it is. эritrokariotsitov, iron-containing pellets. These granules of iron surrounded the core ring, besides them erythrokaryocytes there is a significant amount of iron, located extracellularly. Due to the presence of these granules disease became known as sideroblastic refractory anemia. Ringed sideroblasts are revealed not only in sideroblastic anemia. They are found in hereditary anemias, related to violation of the synthesis of porphyrin, anemia, due to lead poisoning, and a deficiency of pyridoxine (Vitamin B6), thalassemia and aplastic anemia. The discovery of a large number of sideroblasts still does not give grounds for diagnosing refractory sideroblastic anemia.
The disease progresses slowly, and a small proportion of patients (about 7 %) a few years from its beginning in the peripheral blood appear sporadic blasts, and then in the terminal period of the picture of myeloid or acute leukemia myelomonoblastic.
However, refractory sideroblastic anemia, not all patients is the beginning of acute leukemia. In some cases it is a somatic mutation, resulting in the inadequate division erythrokaryocytes, gemoglobinizatsiya lack these cells with the appearance of hypochromic erythrocytes, inadequate division of neutrophilic granulocytes, whereby a shift occurs stab, appearing cells, reminding pelgerovskuyu anomaly of leukocytes.
It can be assumed, somatic mutation that causes a change in the chemical form of iron in the mitochondria and consequently a violation of its inclusion in the hemoglobin molecule.
In some cases, a similar clinical picture and the morphological may arise as a result of somatic mutation, and due to the presence of autoantibodies, directed to the antigen erythrokaryocytes. In most cases, antibodies lead to the disappearance or reduction of red marrow germ, However, a number of patients in a certain period of time or permanently antibody "knock out" a oxyphilic normocytes and reticulocytes, while the number of basophilic and polychromatic normocytes increased in the bone marrow. Due to inefficient erythro- Poetry amount of iron in the bone marrow increased. Correct diagnosis helps identify antibodies, directed against an antigen erythrokaryocytes (same, how and partsialynoy krasnokletochnoy aplasia).
