[Autoantibodies similar to anti-differential ones in immune neutropenia].
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Biomedical subjects
Publications and source records attributed to E A Mamedova.
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Investigations were conducted in 4 infants with alloimmune neutropenia caused by leuko-agglutinins (2 cases) and granulo-cytotoxins (2 cases) detected in the mothers' and infants' sera. Anti-granulocytic antibodies reacted with granulocytes of the child and father but did not react with the mother's own cells. A more severe clinical course (repeated pyo-inflammatory diseases, sepsis) was recorded in infants with alloimmune neutropenia caused by granulo-cytotoxins, alloimmune neutropenia was characterized by disorders in neutrophil phagocytic activity (mainly, due to decreased digestive capacity of cells), inhibition of colony-forming capacity of precursor-cells of granulocytopoiesis; a tendency to T-lymphocytopenia was noted during the study of cellular immunity parameters. Prognosis was favourable in all the cases of neutropenia. The maximum term of neutropenia duration was 6 months. The catamnesis has shown that the development of the infants is normal and they fall ill not often.
The content of hemopoietic and stromal precursor-cells was studied in the bone marrow of 46 children with congenital neutropenia and of 2 children with chronic benign neutropenia. It was found that the number of GM-CFC and F-CFC in the bone marrow of patients with chronic benign neutropenia did not differ from that in the control group of normal children, and the lowering of the neutrophil number in the blood was, probably, associated with their redistribution mechanism or increased destruction in the body. Multiple defects of hemopoietic and stromal tissue were detected in children with a hereditary form of congenital neutropenia when anomalous proliferation of F-CFC and disorders in GM-CFC differentiation led to hypoplasia of granulocytic growth stem and neutropenia.
The study of precursor cells of granulocytes and macrophages has shown that in children with immune neutropenia the higher division of granulopoiesis-committed precursor cells is not affected, while the defect is localized in the periphery of hemopoiesis, and it is induced by increased destruction of neutrophils.
Physical characteristics of bone marrow cells of normal donors were comparatively studied with those of children with immune neutropenia. As a result of the bone marrow cell separation in the density gradient according to their sedimentation rate, fractions enriched with cells of one histogenetic series (lymphoid, erythroid and granulocytic) were obtained. Electrophoretic mobility of immature granulocytes in normal donors differed from that in children with immune neutropenia.
The clinical course and some immunohematologic parameters of the blood and bone marrow were investigated in chronic neutropenia children with a high break of granulocyte maturation. Heterogeneity of etiopathogenetic mechanisms of the disease has been recorded in this group of children.
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The previously established fact of low activity of Ca, Mg-dependent endonucleolysis of cell nucleus DNA in lymphoproliferative diseases (CME-activity) brought the authors to study intranuclear characteristics of lymphoid cells in childhood acute lymphoblastic leukemia (ALL). The intensity of DNA-endonucleolysis was measured in 0.7% agarose gel using electrophoresis. Peripheral blood and bone marrow samples from 13 untreated ALL patients and 23 children in remission were examined. The age of the patients ranged from 4 to 14 years. CME-activity before treatment appeared to be 2-10 times less than normal in 8 out of 13 patients. In bone marrow cell nuclei CME-activity was universally reduced 3-20-fold. In ALL remission endonuclease activity in blood and bone marrow cells returned to normal.
The authors investigated cellular and humoral immunity in 53 children over 3 years of age suffering from acute lymphoblastic leukemia. The children had remission lasting from 6 to 120 months and were followed up for 7-14 years after the diagnosis was made. The treatment was performed according to programs of polychemotherapy practiced in 1981-1988. In November of 1995 42 children were alive, 15 had the disease for 10 years. Lymphocytopenia (absolute number of T-cells and B-cells fell 3-5 and 2-3-fold, respectively) was reported in all the examinees both in early remission and later (6-12, 24-60, 60 and more months since the disease onset). In early remission there was a significant reduction in the serum IgG, IgA and IgM. In children with ALL lethal outcome serum IgM and absolute number of E-RFCa dropped in early remission more significantly indicating deep drug-induced depression of lymphocytopoiesis. After 5 years of treatment the pool of peripheral T-lymphocytes and T/B lymphocyte proportion changed for the best, though their absolute number was subnormal. Serum IgG, IgA and circulating immune complexes were 1.3-1.5 times higher than normal which may be explained by gastrointestinal pathology and food allergy in the majority of children treated.
Peripheral blood (PB) neutrophils and lymphocytes from 14 patients with immune neutropenia were studied using the technique of rosette-formation in 2 neutrophil fractions with diverse specific density. All the neutropenia cases were divided into 2 groups by phagocytosis completeness in all the PB neutrophil types studied. Patients of group 1 had impaired phagocytosis D-RFN in elevated levels of these cells, autorosette-forming lymphocytes and neutrophils. Patients of group 2 had defective phagocytosis D-RFN and EAC-RFN in combination with extremely high contents of autorosette-forming including early lymphocytes. The above complex of techniques may be used for prediction of the duration and severity of neutropenia which is important for further selective studies into the causes of neutropenia and effective treatment.