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Biomedical subjects

I Quinti

Publications and source records attributed to I Quinti.

At least 73 records · Page 4Linked to original sources

A study of IgE in immunoglobulin preparations for intravenous administration. I. IgE in intravenous IgG.

We have studied the IgE content of different lots of commercial intravenous IgG preparations used in our day hospital as replacement therapy for patients with agammaglobulinemia. Two enzymatic methods were used: Phadezym and FAST. The average amounts of IgE detected in Endobulin and Sandoglobulin lots were very high with respect to serum values in the general population. In some lots we found low titers of specific IgE (RAST class 1) to house dust mite, rye grass and cow's milk. We also found in most preparations the presence of IgG anti-IgE which are usually present in sera from atopic patients. Despite a low post-infusion increase of serum IgE and the absence of sensitization or adverse reactions observed, a careful selection of donors with normal IgE levels may need to be recommended to manufacturers in the future.

Agammaglobulinemia↗

[In vitro effects of lysozyme on some specific and non-specific functions of the immune system].

In this paper the in vitro effects of lysozyme on several immunological function was investigated. Henn-egg-white lysozyme (at concentrations ranging between 1 and 10 micrograms/ml) has shown an inhibitory action on mitogen-induced lymphoblastigenesis and autologus mixed lymphocyte reaction. On the other hand, NK activity against K 562 cell line resulted to be increased in the presence of lysozyme. In addition lysozyme has shown to behave as chemokinetic factor increasing PMN random locomotion and inhibiting PMN response only towards complement derived chemotaxins, whereas there was no effect against other chemotactic factors. Then, in addition to its enzymatic activity, a role of lysozyme as modulator of the inflammatory response may be proposed.

Adult↗

Immunoglobulin deficiencies and meningococcal disease.

Five male military recruits were studied for evidence of immunologic deficiencies. Four had recovered recently from a first-episode meningococcal meningitis and one presented with recurrent meningococcemia. A selective decrease in IgG and IgA was found in one of the first-episode patients and a selective IgG2 subclass deficiency in the recurrent case. No complement abnormalities were detected, however, in any of the five cases. These results present further proof of the crucial role of immunoglobulins in the defense against N. meningitidis and they stress the necessity of a through examination of humoral immunity before military career enlistment.

Adult↗

Recurrent sinopulmonary infection and impaired antibody response to bacterial capsular polysaccharide antigen in children with selective IgG-subclass deficiency.

We studied 20 children with recurrent sinopulmonary infections and serum IgG levels within the normal range, who had selective IgG-subclass deficiency. Twelve of the children were IgG2 deficient, five were IgG3 deficient, and three were deficient in both IgG2 and IgG3. IgA deficiency was present in 3 of the 20 patients. In the children with IgG2 deficiency, serum antibody concentrations to the capsular polysaccharide of Hemophilus influenzae type B (Hib) were significantly lower than those in age-matched controls, both before and after immunization with the Hib capsular polysaccharide antigen, which elicits antibody predominantly of the IgG2 subclass. In contrast, their serum antibody titers to the tetanus and diphtheria toxoid protein antigens, which elicit antibody predominantly of the IgG1 subclass, were normal in comparison with those of age-matched controls. These results suggest that impairment of the antibody response to specific microbial antigens predisposes patients with selective IgG-subclass deficiencies to recurrent infections. Thus, as an aid in determining therapy, children with recurrent infections and normal total serum IgG should be evaluated for this condition.

Adolescent↗

Acquired C1-inhibitor deficiency associated with antiidiotypic antibody to monoclonal immunoglobulins.

The syndrome of acquired angioedema and C1-inhibitor deficiency is associated with B-cell lymphoproliferative disease. It is characterized by accelerated consumption of C1q and C1 inhibitor in vivo and by low levels of serum C2 and C4. Four patients with B-cell malignant diseases (IgA myeloma, macroglobulinemia, chronic lymphocytic leukemia, and B-cell lymphoma, respectively) and acquired C1-inhibitor deficiency were found to have circulating antiidiotypic antibodies to the monoclonal immunoglobulin expressed on the surface of their B cells (three patients) or in the cytoplasm of their bone-marrow cells (one patient). Two of the four patients had circulating M components, and their antiidiotypic antibodies reacted with the M components. In three patients studied the percentage of B cells bearing C1q was 18, 24, and 35 per cent, as compared with 2.3 +/- 1.7 per cent (mean +/- S.D.) in six normal controls. These results suggest that an interaction between the idiotype of monoclonal immunoglobulins and antiidiotypic antibodies causes increased consumption of C1q and C1 inhibitor in patients with acquired angioedema and C1-inhibitor deficiency. We propose that the subsequent activation of the early components of complement leads to increased vascular permeability and to angioedema and that these patients have a disease caused by antiidiotypic antibodies.

Aged↗

Thymopentin treatment of selective IgA deficiency.

Thymic hormones have been shown to modulate immunoglobulin production in a number of experiments and it is generally agreed that this action is mediated by modulation of helper and/or suppressor T cell activities. The possibility of upregulating the immunoglobulins is of particular relevance in patients with hypogammaglobulinemias and this paper reports on the results of thymopentin treatment in 9 patients with selective IgA deficiency. Two out of 4 patients responded positively in an open-label trial; in one the serum IgA values remained stable up to 8 weeks after discontinuation of treatment whereas there was a rapid fall in the other. Both responders had consistently normal T4/T8 ratios during the treatment, whereas the nonresponders revealed high ratios with large fluctuations of the T4/T8 ratio. In a subsequent (still ongoing) double-blind trial in 5 patients (3 thymopentin, 2 placebo) no significant change of serum or secretory IgA levels has been observed. Taken together, the data suggest that the tested dose regimen of thymopentin (i.e. daily i.m. injections of 1 mg/kg for 2 weeks, then same dose 3 time weekly for 10 weeks) may only work in a subset of patients with selective IgA deficiency. In the present study we did not attempt to distinguish to which of the three known subgroups the 9 patients belonged, nor did we try alternative dose regimens of thymopentin.

Adjuvants, Immunologic↗

Cultured T cells from patients with T cell chronic lymphocytic leukemia demonstrate a normal phenotype.

Peripheral blood mononuclear cells from eight patients with the rare T-cell form of chronic lymphocytic leukemia were isolated and cultured with Interleukin-2 (IL-2). In all but one case, cultured T-cells (CTC) were established. Various culture conditions were tested for their effectiveness; feeder layers proved valuable for expanding the cultures to large volumes. The CTC remained IL-2 dependent. Analysis of surface determinants on these CTC showed a polyclonal proliferation of T-cells. The distribution of subset markers in the patients' CTC population had completely changed in comparison to the "fresh" peripheral blood cell population but was similar to CTC initiated from healthy donors. Our data suggest that the patients' few contaminating normal T-lymphocytes expanded in culture, while the malignant cells were unresponsive to IL-2. This conclusion is supported by growth characteristics and morphology of the CTC.

Antigens, Surface↗

Chromosome studies in patients with T-CLL chronic lymphocytic leukemia and expansions of granular lymphocytes.

Chromosome analyses were carried out in eight patients with lymphoproliferative disorders of mature T and NK cells. Three cases were characterized by an abnormal expansion of granular lymphocytes (GL), one by a lymphoma of GL with leukemic spread, and four by an OKT4-T-CLL. In four patients cytogenetic studies were performed on bone marrow cells; in seven patients peripheral blood lymphocytes were examined by either direct preparations or PHA-stimulated cultures. Six patients displayed a normal karyotype. Two cases belonging to the OKT4-T-CLL group had a chromosome number ranging from 44 to 47, with multiple numerical and structural clonal anomalies. Clonal anomalies could be a feature of patients with the more aggressive clinical course.

Adult↗

Characterization of two patients with lymphomas of large granular lymphocytes.

Two patients with non cutaneous well-differentiated lymphocytic lymphoma with leukemic spread are reported. The large majority of their peripheral blood mononuclear cells (PBMC) formed rosettes with sheep erythrocytes, had receptors for the Fc portion of IgG, and an enzymatic profile of relatively mature T-cells. These cells were morphologically characterized as large granular lymphocytes. Studies with monoclonal antibodies in one of the cases showed an OKT3+, OKT10-, OKT4-, OKT8-, HNK-1-, OKM1+ phenotype, whereas PBMC from the other case were OKT3+, OKT10-, OKT4-, OKT8+, HNK-1+, OKM1-. PBMC from the first patient were able to suppress in vitro B-cell differentiation and were capable of a strong antibody dependent cellular cytotoxicity (ADCC) activity. Natural killer (NK) activity was reduced. Cells from the other patient who was hypogammaglobulinemic, exerted suppressor activity in immunoregulatory assays, and showed ADCC and NK activity. These data support the existence of LGL lymphomas consisting of the proliferation of mature appearing cells capable of functional activity.

Antibodies, Monoclonal↗

Unusual phenotype (Leu 7+, OKT4+, OKM1+) expressed by cells from a patient with an abnormal expansion of granular lymphocytes.

We report the case of a 70-year-old female with a lymphocytosis which was casually detected during a routine examination. Immunological studies revealed the expansion of granular lymphocytes (GL) with the following, previously undescribed phenotype: Leu 7+, OKT3+, OKT4+, OKT8-, OKM1+. These cells were tested for their functional activities and found to exert neither helper nor suppressor functions in in vitro tests. Cytotoxic activities demonstrated a strong ADCC and a markedly reduced NK function. 1 year later the clinical course has remained good without any treatment and we suggest that this case should be classified as an abnormal expansion of GL, despite the OKT4 positivity of the cells. Our data point out the importance of a careful immunological study of cells from these rare patients and suggest the existence of a normal GL population expressing the OKT4 phenotype, which is possibly expanded in this patient.

Aged↗

Abnormal expansions of polyclonal large to small size granular lymphocytes: reactive or neoplastic process?

Morphological, immunologic, and functional properties of peripheral blood cells from two patients with chronic proliferations of granular lymphocytes are described. Cells from both patients showed a heterogeneous pattern from both a morphological and immunologic standpoint, indicating a polyclonal, rather than a monoclonal, expansion of these cells. In fact, both large and small-to-medium-sized granular lymphocytes were observed, and different percentages of positivity were found in the analysis with a large panel of monoclonal antibodies. Serologic and histologic features support the hypothesis that this lymphocytosis could be secondary to bacterial or viral infections rather than a primary event, suggesting that these patients may have chronic reactive immunoregulatory disorders.

Acid Phosphatase↗

Thymopoietin pentapeptide treatment of primary immunodeficiencies.

26 patients with primary immunodeficiencies (3 infants with severe combined immunodeficiency [SCID] 3 with DiGeorge syndrome, 6 with T-cell defect or SCID with B cells, 4 with common variable hypogammaglobulinaemia and associated T-cell defect, 5 with ataxia-telangiectasia, and 5 with hyper-IgE syndrome) were treated with thymopoietin pentapeptide (TP-5) at a dose of 0 . 5 mg/kg daily for 2 weeks and then 3 times a week at 0 . 5 mg/kg for 10 weeks, 3 patients with DiGeorge syndrome and 3 with primary T-cell defect demonstrated pronounced clinical and immunological improvement during treatment. None of the patients with SCID and 3 of 6 patients with SCID with B cells or primary T-cell defect showed any clinical or immunological changes during therapy. In 5 patients with ataxia-telangiectasia clinical manifestations and immunological tests were unchanged by TP-5. Abnormality of T cells in cases of hyper-IgE syndrome was not corrected by TP-5 treatment.

Adolescent↗

Defect of T helper lymphocytes, as identified by the 5/9 monoclonal antibody, in patients with common variable hypogammaglobulinaemia.

Peripheral blood lymphocytes from 17 patients with common variable hypogammaglobulinaemia (CVH) were tested for reactivity with the 5/9 monoclonal antibody which reacts with about 15% of normal T-PBL in which all helper activity is found. In PBL from CVH patients, the proportions of OKT4 and OKT8 positive cells were also determined. Five patients had normal percentages of 5/9 cells and a normal OKT4/OKT8 ratio. Twelve patients had significantly decreased (or absent) 5/9 lymphocytes. Among these, five had decreased 5/9 cells and a normal OKT4/OKT8 ratio and seven had decreased 5/9 cells and an inversion of the OKT4/OKT8 ratio. The deficiency of the helper phenotype T cell subpopulation identified by the 5/9 monoclonal antibody in many patients with CVH may be relevant in the pathogenesis of this disease.

Adolescent↗

Usefulness of monoclonal antibodies in the diagnosis and monitoring of patients with primary immunodeficiencies: combined experience in three clinical immunology centers.

Circulating levels of T-cell subsets and NK cells were determined in 78 patients with primary immunodeficiencies, 35 children with recurrent respiratory infections, and healthy age-matched controls. Normal T cell and natural killer (NK) cell values were observed in individuals with immunoglobulin A (IgA) deficiency and X-linked agammaglobulinemia, while reduced OKT4/OKT8 cell ratios and low levels of 5/9+ T helper cells were found in approximately 60% of patients with common variable immunodeficiency. Infants with severe combined immunodeficiency (SCID) and lymphopenia had virtually no cells expressing T-cell or NK-cell surface antigens, but had normal numbers of monocytes and other types of blood cells. Infants with DiGeorge syndrome, other primary T-cell defects, or SCID with B cells had few or no circulating cells of mature T helper-suppressor phenotypes, but had normal numbers of NK cells (HNK-1+) and NK function. These results support the idea of a common stem cell precursor for T, B, and NK cells, each of which follows a separate pathway of differentiation. Profound alterations were observed in the distribution and function of T-cell subsets in ataxia-telangiectasia patients who were previously shown to have thymic dysplasia. A significant reduction in the frequencies of OKT3+ and OKT4+ cells was observed in children with frequent respiratory infections during infancy.(ABSTRACT TRUNCATED AT 250 WORDS)

Agammaglobulinemia↗

T-dependent immunity in aged humans. II. Clinical and immunological evaluation after three months of administering a thymic extract.

We investigated the effect of Thymostimulin (TP-1) (a bovine thymic extract) on some clinical and immunological parameters in hospitalized aged humans. Forty aged patients were randomized into two groups, one received no therapy and the other was treated with TP-1 for 3 mth. The Ig and complement levels, sheep rosette-forming cells, absolute lymphocyte numbers and sedimentation rate were measured on days 0, 90 and 180. Moreover, the incidence of infections was determined. The baseline immunological data of the patients studied were within the normal range. These data remained unchanged after TP-1 treatment. In the TP-1 treated group we observed a significant reduction of infections (P=0.023) in comparison to the control group. A significant reduction of the sedimentation rate was also observed in the treated group.

Aged↗