[Histiocytic syndromes in children: the classification problems and diagnostic criteria].
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Biomedical subjects
Publications and source records attributed to N A Finogenova.
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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.
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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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Peripheral blood and bone marrow morphology, blood and bone marrow lymphocyte subpopulation composition were studied in two children with cyclic neutropenia, using flow cytofluorometry, monoclonal antibodies, colony-forming capacity of granulocytic macrophagal precursors in semi-fluid agar. The studies were conducted in varying periods of the neutropenic cycle. Differences were revealed in immunohematologic parameters and clinical course of neutropenia in the two patients. The analysis of the literature data and the authors' own observations of the patients with cyclic neutropenia permitted a suggestion on high heterogeneity of pathogenetic mechanisms of this disease.
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