Concept dizeritropoetichesky anemias integrate anemic syndromes, heterogeneous on the genesis, which general signs are the inefficient erythrogenesis and qualitative changes of erythroidal elements generally in the form of disturbances of division of eritrokariotsit.
Epidemiology Inborn dizeritropoetichesky anemias meet exclusively seldom, acquired more often.
Etiology and pathogeny Inborn forms of a disease are inherited on autosomal recessively type. The acquired (secondary) dizeritropoetichesky anemias can meet at deficit of hemopoietic factors, antitubercular treatment, some intoxications.
The inefficient erythrogenesis is first of all disturbance of kinetics, eritro-and normoblasts therefore at their sufficient quantity in marrow anemia, a reticulocytopenia develops. Predecessors of erythrocytes because of their qualitative inferiority collapse directly in marrow. Perhaps defeat of a stem cell and (or) its microenvironment is the reason of products of such cells.
The inefficient erythrogenesis occurs also at healthy faces which have about 5 — 10 defective % eritro-and normoblasts also perish in marrow. However in physiological conditions the main erythrogenesis quite offsets such loss.
Classification Allocate the inborn and acquired (secondary) forms of dizeritropoetichesky anemias.
Approximate formulation of the diagnosis: 1. Dizeritropoetichesky anemia (option I) with megalosblastoidny type of a hemopoiesis, a moderate hyperbilirubinemia, insignificant increase in a spleen, a macrocytosis and an anisocytosis of erythrocytes. 2. Dizeritropoetichesky anemia (option II) with multinuclear erythroblasts, a splenomegaly, increase in a liver with the phenomena of cirrhosis and hemochromatosis. 3. Dizeritropoetichesky anemia (option III) with huge multinuclear erythroblasts in marrow, internuclear chromatinic bridges and the phenomena of a karyorrhexis.
Clinic Signs of a dizeritropoez come to light in the form of disturbance of synchronization of a kernel and cytoplasm, its vacuolation and a basophilia, excess content of iron in lysosomes and mitochondrions; changes of the sizes of kernels, their structure and a form, two - or multinuclear erythroblasts between which there are "bridges" connecting them, fragmentations of a kernel as a result of a karyorrhexis and pycnosis, quite often signs of a megaloblastny erythrogenesis.
Level of transport iron of blood serum is increased or normal. The considerable siderosis of various bodies is noted. The lysis of erythroidal cells in marrow is followed by a moderate bilirubinemia (at the expense of free fraction), duration of their life is usually shortened. In cases of the dizeritropoetichesky anemias which are combined with a dysgranulocytopoiesis anomaly of nuclear segmentation of neutrophils, sometimes changes of a karyotype, disappearance of specific granularity of granulocytes is observed. In the degranulated neutrophils there are no peroxidase and an alkaline phosphatase. By electronic microscopic examinations it is established that azurophilic granularity does not contain peroxidase in promyelocytes. Distinguish separate options from inborn dizeritropoetichesky anemias.
The option I differs in signs of a megaloblastny hemopoiesis, a macrocytosis, two-nuclear erythroblasts with chromatinic "bridges" between them, existence of sideroblasts, but not ring-shaped forms. Anemia and hyperbilirubinemia moderate. Small increase in a spleen. Quantity of leukocytes and thrombocytes usually normal. The reticulocytosis is unsharply expressed. Erythrocytes are generally provided by macrocytes with an anisocytosis. The considerable hyper siderosis of bodies takes place.
The option II is characterized by the multinucleosis of erythroblasts which is combined with positive acid and serumal test. Anemia is usually normotsitarny, it is moderately expressed. It is noted gepato-and a splenomegaly, in a liver — the phenomena of cirrhosis and hemochromatosis. Erythrocytes a complement are sensitive, but it is many times less, than at Markiafava's illness — Mikeli. The positive test of a lysis of Cold-reactive antibodies comes to light. Sometimes the slabopolozhitelny acid test drops out, but it differs from that at a paroxysmal night haemoglobinuria as it is not found in own serum of the patient. Saccharose test negative. Believe that erythrocytes bear the antigen which is not found normal and at a paroxysmal night haemoglobinuria. Antibodies belong to the class IgM and the positive test is caused by existence on erythrocytes of antigen which reacts with IgM. It is supposed that existence of these antibodies on erythroblasts interferes with normal division of cytoplasm, the kernel shares usually. Multinucleate cells are so formed. They become qualitatively defective and prematurely collapse in marrow.
The option III is characterized by existence in marrow huge multinuclear (to 12 kernels) erythroblasts. The main line is also existence of internuclear chromatinic bridges and the phenomenon of a karyorrhexis. At research of a ferrokinetika signs of a dizeritropoez, studying with 51Cr come to light find small shortening of life of erythrocytes with some increase of their sequestration in a spleen.
The acquired dizeritropoetichesky anemias can be observed at patients with deficit of B12 vitamin, folic acid, iron, at infectious processes, sideroblastny anemias, a thalassemia, aplastic anemia, a myelofibrosis, an Osler's disease, a paroxysmal night haemoglobinuria, at use of antituberculous remedies, intoxication lead. Dizeritropoez, as well as dizgemopoez, sometimes precedes development of a leukosis and (or) accompanies its current.
Verification of the diagnosis Diagnosis of inborn dizeritropoetichesky anemias is based on noted features inherent to these forms. Diagnosis of the acquired (secondary) dizeritropoetichesky anemias demands a specification of a basic disease, determination of deficit of hemopoietic factors against which there can be characteristic disturbances of a hemogenesis. At an outcome of dizeritropoetichesky anemia in an acute miyeloblastiy leukosis in marrow punctate the miyelokariotsitopeniya sometimes comes to light that causes the necessity of histologic studying of a bioptat of an ileal bone for a hemopoiesis hypoplasia exception.
Treatment Tactics of therapy of dizeritropoetichesky anemias remains still undeveloped. At a deep anemia appoint the transfusion of the eritrotsitny weight (defrozen, washed). The acquired dizeritropoetichesky anemias can be reversible in cases of elimination of the reasons which caused their development. There are separate messages on use of a splenectomy at hereditary forms of a disease.