[Cyclic neutropenia: a disease or a syndrome?]. / Tsiklicheskaia neitropeniia, zabolevanie ili sindrom!

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.

[Phenotyping of blood and bone marrow lymphocytes in children with primary immunologic deficiency]. / Fenotipirovanie limfotsitov krovi i kostnogo mozga u detei s pervichnoi immunnoi nedostatochnost'iu.

Subpopulational composition of peripheral blood and bone marrow lymphocytes was studied in 17 children with different forms of immunodeficiency, combined with hematological shifts (agammaglobulinemia--6, mucocutaneous candidiasis--2, selective IgG-deficiency--2, hyper-IgM syndrome--3, cephalo-oculocutaneous telangiectasia (COCT)--2, general, variable immunodeficiency--2 patients; neutropenia was observed in all the patients, lymphopenia--in 13, anemia-in 6 patients. Surface markers were assayed by flow cytofluorometry with monoclonal antibodies OKT3, OKT4, OKT8, OKB7, produced by "Ortho diagnostics". Changes characteristic of certain forms of primary immunodeficiency have been revealed in the subpopulational composition of peripheral blood and bone marrow lymphocytes: decreased helper potential in patients with general variable immunodeficiency, T-lymphocyte deficiency in patients with COCT increased number of phenotype T3 cells and decreased amount of B-cell in agammaglobulinemia patients. Significant heterogeneity has been noted in the parameters of hemogram, myelogram and in the subpopulational composition of peripheral blood and bone marrow lymphocytes in each nosologic form, the group of patients with hyper-IgM syndrome has proved to be most heterogeneic. It has been suggested that the changes in the subpopulational composition of bone marrow lymphocytes may be responsible for primary immunodeficiency and disorders in hemopoiesis.

[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.

[Membrane markers of T- and B-lymphocytes in children with primary immunologic deficiency conditions]. / Membrannye markery T- i B-limfotsitov pri pervichnykh immuno- defitsitnykh sostoianiiakh.

Altogether 79 children with primary immunodeficiency conditions (PIC) and 21 children suffering from frequently occurring bacterial infections were examined for the level of rosette-forming cells and lymphocytes reacting positively with monoclonal antibodies to different phenotypic membranous markers (SD-antigens and others). The patients with combined forms of immunodeficiency, with T deficiency playing the key role manifested a significant lowering of the content of the subpopulations of SD3+, SD5+, SD7+ and SD4+ lymphocytes and the decrease of the SD4/SD8 ratio. The the reduction of the number of B lymphocytes (smIg + K1, SD19 +), the low levels of Ia+ -cells, and the decrease of the SD4/SD8 at the expense of the content of SD8+ -cells. In the patients with selective immunodeficiency and recurrent bacterial infections without verified PIC, these parameters did not undergo any essential changes. As compared with immunofluorescent assays, the measurement of E-RFL and M-RFL amounts was found to have insufficient information content for the diagnosis of PIC.

Vaccinia virus persistence in a child against the background of immune deficiency.

A young girl, vaccinated against smallpox 6 years before suffered from a persistent vaccinia virus infection and a congenital skin disease, i.e. epidermolysis bullosa. The virus was isolated from skin lesions at the vaccination site and remote sites and repeatedly from the blood; it was not isolated from bone-marrow specimen, saliva, pharynx or urine. The titre of virus-neutralizing antibodies was low (1:10), immunoglobulins A, M and G were within age-related limits; antibodies against measles and tetanus were at protective levels, skin tests were positive. Staphylococcal antitoxin titre was extremely high. The child's mother, not vaccinated against smallpox, possessed vaccinia--virus-neutralizing antibodies at high titre (over 1:320). Examination of the child did not show any quantitative immune deficiency. Immune deviations were found in the lymphocyte blast-transformation reaction on stimulation with PHA and specific antigen, as well as in the nonspecific-suppression test. The possible genesis of the virus persistence and the role of the virus in the clinical course of the disease are discussed.

[Complex immunochemical analysis of the proteinogram and the system of soluble leukocytic antigens in children with chronic and recurrent infections]. / Kompleksnyi immunokhimicheskii analiz proteinogrammy i sistemy rastvorimykh antigenov leikotsitov u detei, stradaiushchikh khronicheskimi i retsidiviruiushchimi infektsiiami.

The immunochemical study of the blood sera of children with chronic and relapsing infections has shown an increase in the content of alpha 2-macroglobulin and alpha 1-antitrypsin in the absence of significant changes in the concentration of immunoglobulins and complement components. The immunochemical analysis of the system of soluble leukocytic antigens (SLA) has revealed a decrease in the level of SLA-1 simultaneously with the presence of redundant amounts of SLA-5 and SLA-8.