[Frequency of opportunistic infections in children with immune neutropenia and their mothers].

AIM: Determine the frequency of opportunistic infections among children with immune neutropenia and their mothers. MATERIALS AND METHODS: 66 mothers and 66 children with immune neutropenia diagnosis were examined for the presence of herpes (HV) and pneumocystic infection. Opportunistic infections markers (IgM, IgG, early and late antigens, virus reproduction) were determined by enzyme immunoassay, immunofluorescence reaction and rapid culture method (vero, u937, human fibroblasts). RESULTS: Pneumocystosis was the most active infection in the group. Among mothers 26 (39.3%) cases of pneumocystic infection in acute form were detected, among children - 18 (27.3%) cases. Infection occurred only in acute form during primary infection; there were no cases of its reactivation, which is an indication of recent pneumocystosis infection. Analysis of data on detection of acute and recent herpes infections showed that HV infection markers were determined in a relatively large number of mothers and their children: herpes simplex virus - 21.2%, Epstein-Barr virus - 12.0%, cytomegalovirus - 15.0%, Human herpesvirus 6 - 10.6%, Pneumocystis carinii - 21.2%. The data provided give evidence on a possible family pattern of the infection. CONCLUSION: A necessity of examination of mothers and their children suffering from immune neutropenia was shown because the specified opportunistic infections can form intra-family foci. The presence of acute form of infection in mother may be one of the conditions of development of this infection in the child.

[Toll-like receptor-mediated functional activity of mononuclear cells in children with neutropenia].

AIM: To study the Toll-like receptor (TLR)-mediated functional activity of peripheral blood mononuclear cells (PBMC) from children with different forms of neutropenia. MATERIALS AND METHODS: TLR-mediated functional activity of PBMC was evaluated by production of proinflammatory cytokines--TNF alpha and IFN alpha. Ligands for TLR1/2, TLR 2/6, TLR4, TLR5, and TLR9 were used to stimulate TNF alpha production by PBMC from healthy children and children with neutropenia. Ligands for TLR3, TLR4, TLR7, TLR7/8, TLR8, and TLR9 were used to induce production of IFN alpha. Levels of TNF alpha and IFN alpha were measured in PBMC supernatants by ELISA. The group of patients with neutropenia included 9 children with immune neutropenia and 3 children with congenital neutropenia. Control group consisted of 12 healthy children of the same age range. RESULTS: It was revealed that TLR2, TLR4, and TLR5 ligands have enhanced stimulating effect on TNF alpha production by PBMC of children with congenital neutropenia and had no effect on PBMC of children with immune neutropenia. Children with immune neutropenia are characterized by significantly increased IFN alpha production induced by ligands of TLR3, TLR8, and TLR9. CONCLUSION: Revealed changes of TLR-mediated functional activity of PBMC from children with various forms of neutropenia may play significant role in development and course of infections in these patients.

[Retrospective evaluation of blood immunologic parameters in the course of remission of acute lymphoblastic leukemia in children]. / Retrospektivnaia otsenka immunologicheskikh pokazatelei krovi u detei v raznye sroki remissii ostrogo limfoblastnogo leikoza.

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.

[Subpopulations of peripheral blood neutrophils and lymphocytes in children with immune neutropenia]. / Subpopuliatsii neitrofilov i limfotsitov perifericheskoi krovi u detei s immunnoi neitropeniei.

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.

[Changes in the activity of endonucleolysis of nuclear DNA in children with acute leukemia]. / Izmenenie aktivnosti éndonukleoza DNK-kletochnykh iader pri ostrom leikoze u detei.

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.

[Alloimmune neutropenia in newborns]. / Alloimmunnaia neitropeniia novorozhdennykh.

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.

[Evaluation of hematopoietic and stromal cells-precursors in children with neutropenia]. / Otsenka krovetvornykh i stromal'nykh kletok-predshestvennikov pri neitropeniiakh u detei.

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.

[Precursor cells of the granulomonocytopoiesis in children with immune neutropenia]. / Kletki-predshestvenniki granulomonotsitopoéza u detei s immunnoi neitropeniei.

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.

[Two-dimensional size-charge distribution of bone marrow cells in children with chronic immune neutropenia]. / Dvukhmernoe raspredelenie razmer-zariad kletok kostnogo mozga u detei s kronicheskoi immunnoi neitropeniei.

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.

[Immunohematologic characteristics and course of chronic neutropenia in children with severe decrease of granulocyte maturation]. / Immunogematologicheskaia kharakteristika i techenie khronicheskikh neitropenii u detei s vysokim obryvom sozrevaniia granulotsitov.

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.