1/20. Strong alpha beta and gamma delta TCR response in a patient with disseminated mycobacterium avium infection and lack of NK cells and monocytopenia.infection with atypical mycobacteria occurs mainly in patients with a compromised cellular immune system, in particular in those with a defective T cell or monocyte function. Here we analyzed the specific immune response of an adolescent hiv-negative patient with disseminated mycobacterium avium infection and fatal varizella zoster virus infection. The patient presented with dysplastic hematopoesis of all cell lineage's and a bicytopenia of erythrocytes and leukocytes, but a hematological malignancy could not be found. We found a peripheral lymphopenia and monocytopenia, as well as a lack of NK-cells and B-cells. Lymphocytes consisted of 95% T cells, which contained up to 40% of TCR gammadelta CD4-CD8-T-cells (mainly TCR gamma9delta2), few monocytes and B-cells. Approximately 50% of CD3 T-cells showed a CD57 NK-like phenotype. Functional analysis of PBMC revealed a good antigen-specific T cell function if antigen-presenting cells were supplemented from a HLA-matched donor. Moreover, a strong M. avium specific cytotoxicity mediated by TCR alphabeta T-cells could be found in vitro and even ex vivo. In contrast, NK-killing was absent. No evidence for a defect in IL-12 or IFN-gamma production and signaling were found. The data indicate that a strong alphabeta and gammadelta T cell immunity tries to compensate for a deficient monocyte and NK cell function in this patient.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/20. Selective T-cell deficiency in Turner's syndrome.The case of a 29-year-old Caucasian woman with 45 X0 karyotype, known as Turner's syndrome, and a recently diagnosed selective T-cell deficiency is reported. The main clinical features of the patient were recurrent sinopulmonary infections and a negative skin test with seven common recall antigens. Laboratory findings included lymphocytopenia, highly elevated CD45RA/CD45R0 ratio, as well as reduced expression of the co-stimulatory molecules CD154, CD86, CD80 and CD28 on CD4 cells in combination with disturbed lymphocyte transformation in vitro. Markedly decreased levels of interleukin (IL)-2R, both on lymphocyte surface as well as the soluble analog, suggest a new form of x-linked immunodeficiency associated with Turner's syndrome.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
3/20. Defective dendritic cell maturation in a child with nucleotide excision repair deficiency and CD4 lymphopenia.We report a case of a combined immunodeficiency (CID) in a child affected by trichothiodystrophy (TTD) characterized by an altered response to ultraviolet (UV) light due to a defect in the XPD gene. The XPD gene encodes a subunit of the transcription factor II H (TFIIH), a complex involved in nucleotide-excision repair (NER) and basal transcription. Our patient showed neurological and immune system abnormalities, including CD4 lymphopenia never previously reported in TTD patients. in vitro immunological studies revealed a marked reduction in T-cell proliferation in response to mitogens and CD3 cross-linking which was partially recovered by the addition of anti-CD28 antibody or exogenous interleukin-2. The patient's T cells displayed alterations in T-cell receptor (TCR/CD3) proximal signalling characterized by marked reduction in Lck kinase activity coupled with a constitutive hyperactivation of Fyn kinase. Despite these alterations, normal levels of Lck and Fyn proteins were detected. The role of antigen-presenting cells (APCs) in the pathogenesis of the T-cell defect was investigated by analysing dendritic cells (DCs) generated from the patient's blood monocytes. In these cells, flow cytometry revealed significantly reduced expression of the CD86 co-stimulatory molecules and HLA glycoproteins. In addition, the patient's DCs showed a decreased ability to stimulate naive T lymphocytes. overall, the results of our study suggest that a defective TFIIH complex might result in alterations in T cells and DC functions leading to a severe immunodeficiency.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
4/20. Cellular immunodeficiency and autoimmunity in long-term mineral oil administration.BACKGROUND: Subcutaneous mineral oil injection is an old-fashioned practice used mostly for cosmetic purposes. infection, ulceration, subcutaneous nodules, and autoimmune activation are among the known adverse effects. Immunodeficiency has not been previously reported in association with mineral oil injection. OBJECTIVE: To report the case of a 43-year-old woman who performed long-term self-administration of mineral oil and was found to have both cellular immunodeficiency and autoimmunity. methods: We performed an immunological evaluation. Throat, induced sputum, urine, and blood cultures were examined for microorganisms. Pelvic computed tomography, inguinal lymph node biopsy, bone marrow biopsy, and liver biopsy were also performed. RESULTS: Laboratory results revealed peripheral lymphopenia, very low absolute numbers of lymphocyte subpopulations, and a markedly impaired lymphocyte proliferative response to mitogens (phytohemagglutinin and concanavalin a) and recall antigens (mumps, candida albicans, purified protein derivative, and tetanus toxoid). The cultures were negative for microorganisms. The pelvic computed tomogram demonstrated areas of diffuse oil-density signals throughout the subcutaneous tissue in the gluteal area and proximal lower extremities, as well as bilateral inguinal lymphadenopathy. A lymph node biopsy specimen showed lipid granulomas and necrotizing lymphadenitis. A bone marrow biopsy specimen demonstrated hypercellular marrow with normal trilineage hematopoesis. Increased serum transaminase levels, hypoalbuminemia, positive anti-extractable nuclear antigen and anti-Ro antibodies, and plasma cells in the liver suggested an autoimmune process. CONCLUSIONS: mineral oil administration may be associated with both cellular immunodeficiency and autoimmunity. patients who have received long-term administration of a foreign substance should undergo a comprehensive immunological evaluation.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/20. Acquired T cell specific deficiency other than acquired immunodeficiency syndrome (AIDS).Evidence of an acquired T cell-specific deficiency distinct from acquired immunodeficiency syndrome (AIDS) in a 63-yr-old Japanese female is provided. Recently, this patients suffered from primary invasive pulmonary aspergillosis. skin tests to purified protein derivative of tuberculin (PPD) and aspergillus antigens were negative. Upon admission to our hospital, her lymphocytes were exclusively unresponsive to T cell mitogens (concanavalin a, phytohemagglutinin, and OKT 3). The level of cells defined by monoclonal antibodies (CD1, CD2, CD3, CD4, WT31, and CD5) was less than 3%. In contrast, no decrease in the number of red blood cells, platelets, neutrophils or B cells was apparent. Five years ago, the patient had a normal white blood cell and lymphocyte count. However, over the following 4 yr, she developed lymphopenia. With medication, her pulmonary disease recovered, while lymphopenia still continued. The levels of immunoglobulins, complements and enzyme activities (adenosine deaminase and purine nucleoside phosphorylase) were normal. Moreover, several tests for hiv (ELISA and Western bolt) were negative suggesting that the T cell-specific deficiency was not a congenital immunodeficiency or AIDS but rather a new type of acquired immunodeficiency.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
6/20. Immune deficiency in CHARGE association.CHARGE association is the sporadic, non-random concurrence of coloboma of the eye, heart anomalies, choanal atresia, Retardation of growth and development, Genitourinary anomalies, Ear anomalies and deafness (CHARGE association). Other abnormalities have also been reported in small numbers of patients with CHARGE association. The molecular basis of the CHARGE association is not clear. The spectrum of CHARGE association anomalies is wide and includes multiple systems. CHARGE association shares features with DiGeorge sequence, but no specific immune abnormalities are identified with the CHARGE association. The present study reports immune defects observed in three patients with CHARGE association. All patients presented with frequent upper and lower respiratory infections. The underlying immune abnormalities differ: one patient has impaired T-cell proliferation and poor antibody response to polysaccharide (pneumococcal) antigens; another has T-cell lymphopenia; and the third has a mild IgG2 subclass deficiency. Their course has so far been benign and they are all managed with prophylactic antibiotics. Although no single abnormality of the immune system is recognized in these patients, immune deficiency is considered among the occasional components of the CHARGE association.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
7/20. Persistent B-cell lymphopenia, multiorgan disease, and erythema multiforme caused by mycoplasma pneumoniae infection.We report a 6-year-old girl in whom mycoplasma pneumoniae infection presenting with erythema multiforme, multiorgan, and hematologic dysfunctions induced a long-standing, marked B-cell lymphopenia. An increase of CD8 lymphocytes was also detected. We suggest that a selective cytotoxic T lymphocyte-dependent B cell lysis and the expansion of super-antigen activated CD8 T cells may account for the multiorgan and hematologic disturbances triggered by M. pneumoniae.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
8/20. A chromosomal breakage syndrome with profound immunodeficiency.The chromosomal breakage syndromes--ataxia-telangiectasia, Fanconi's anemia, and Bloom's syndrome--are associated with growth failure, neurologic abnormalities, immunodeficiency, and an increased incidence of malignancy. The relationship between these features is unknown. We recently evaluated a 21-year-old female with more severe chromosomal breakage, immunodeficiency, and growth failure than in any of the mentioned disorders. As of November 1985, the patient remains clinically free of malignancy. At age 18, the patient's weight was 22.6 kg (50th percentile for seven years), height was 129 cm (50th percentile for eight years), and head circumference was 42 cm (50th percentile for six months). Laboratory studies demonstrated a marked decrease in both B and T cell number and function. The peripheral blood contained 400 to 900 lymphocytes/microL with 32% T11 cells, 17% T4 cells, and 21% T8 cells. The proliferative responses to phytohemagglutinin (PHA), pokeweed mitogen, and concanavalin a were less than 10% of control. There were 1% surface IgM positive cells, and serum IgG was 185 mg/dL, IgM 7 mg/dL, IgA 5 mg/dL. In lymphocyte cultures stimulated with the T cell mitogens PHA, phorbol ester, and interleukin 2, 55% of the banded metaphases demonstrated breaks or rearrangements. The majority of the breaks involved four fragile sites on chromosomes 7 and 14, 7p13, 7q35, 14q11, and 14q32. These are the sites of the genes for the T cell-antigen receptor and the immunoglobulin heavy chain and are sites of gene rearrangement in lymphocyte differentiation. Epstein-Barr virus stimulated B cells and fibroblast cultures also demonstrated a high incidence of breaks, but the sites were less selective. These findings suggest that the sites of chromosomal fragility in the chromosomal breakage syndromes may be informative and that factors other than the severity of the immunodeficiency or the high incidence of chromosomal damage may contribute to the occurrence of malignancy in the chromosomal breakage syndromes.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
9/20. T lymphocyte disorder after capnocytophaga ochracea endocarditis.capnocytophaga species are gram-negative rods which may cause disease in both non-immunocompromised and immunocompromised hosts. We describe a case of endocarditis due to capnocytophaga ochracea in a non-immunocompromised patient with a decrease of blood CD4/CD8 ratio and lymphocyte proliferative response to ConA during infection. in vitro experiments showed that C. ochracea decreased lymphocyte proliferation to mitogens (ConA, PHA), cell surface CD4 antigen and IL2 receptor expression on peripheral blood mononuclear cells (PBMC) from normal volunteers.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
10/20. Angioimmunoblastic lymphadenopathy: a T-cell deficiency.The case of a 49-year-old man with the diagnosis of angioimmunoblastic lymphadenopathy is reported. The patient survived a stormy clinical course. The corticosteroids improved dramatically the clinical picture although the patient developed a staphylococcal septicemia. Before treatment immunological studies were done including quantitation of B and T-cells, antigen stimulation of lymphocytes in vitro, skin tests and skin window. Impairment of cell mediated immunity, decreased t-lymphocytes and increased b-lymphocytes were found. A decreased migration of lymphocytes in the skin window was also found compatible with immunosuppression. A possible presumptive pathogenetic mechanism is described although the cause of this recently described entity remains unknown.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
| | Next -> |