1/10. Neutrophil antigen 5b is carried by a protein, migrating from 70 to 95 kDa, and may be involved in neonatal alloimmune neutropenia.BACKGROUND: Neutrophil antigen 5b has been described as involved in transfusion reactions and not in neonatal alloimmune neutropenia. CASE REPORT: Anti-5b was found in the serum of a mother of a persistently neutropenic newborn, who had several bacterial infections. The neutropenia responded to treatment with recombinant human granulocyte-colony-stimulating factor. immunoprecipitation experiments performed with this and three other 5b antisera identified a protein, migrating from 70 to 95 kDa, as carrier of 5b. The observed pattern of migration may point to heavy glycosylation of this protein. RESULTS: Six 5b-negative donors were identified among 54 screened white donors, for a 5b gene frequency of 0.66. CONCLUSION: Alloimmunization to 5b in pregnancy is rare. In the patients with neonatal neutropenia analyzed in the last decade, this was the first case discovered.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/10. 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 = 0.2keywords = antigen (Clic here for more details about this article) |
3/10. Immune thrombocytopenia more than a year after allogeneic marrow transplantation due to antibodies against donor platelets with anti-PlA1 specificity: evidence for a host-derived immune reaction.We report on a male patient transplanted from his HLA-matched sister for Ph1-chromosome positive chronic myelogenous leukaemia who developed immune thrombocytopenia more than 1 year after transplantation. The platelet antibody reacted with the platelet specific antigen PlA1 on donor platelets, and also on recipient platelets after engraftment. A presumed host-versus-donor induced thrombocytopenia was supported by Southern blot analysis using a Y-chromosome specific probe demonstrating residual host-origin cells in the patient's excised spleen.- - - - - - - - - - ranking = 0.2keywords = antigen (Clic here for more details about this article) |
4/10. Familial occurrence of systemic sclerosis, rheumatoid arthritis and other immunological disorders: report of two kindreds with study of hla antigens and review of the literature.We report two Caucasian families with systemic sclerosis and other connective tissue and immunological disorders, including rheumatoid arthritis, discoid lupus erythematosus, psoriasis, psoriatic arthritis, ankylosing spondylitis, ulcerative colitis, asthma, sjogren's syndrome, Raynaud's phenomenon and thyroid disease. In one of these families, two sisters are affected with systemic sclerosis. Clinical, serological, and HLA haplotype results are reported, along with a review of the medical literature on familial occurrence of systemic sclerosis.- - - - - - - - - - ranking = 0.8keywords = antigen (Clic here for more details about this article) |
5/10. Absence of complement receptor type 3 and lymphocyte function antigen 1 causing deficient phagocyte and lymphocyte functions.We describe a patient with delayed umbilical cord detachment, recurrent bacterial infections, and inability to form pus, despite persistent leucocytosis. Immunofluorescence studies with specific monoclonal antibodies showed a severe deficiency in the expression of alpha-chains of the receptor for the C3bi fragment of C3, complement receptor type 3, and the lymphocyte function antigen 1 molecule, found on neutrophil, monocyte and lymphocyte membranes. These membrane antigen defects were responsible for abnormalities in adhesive cell functions. Polymorphonuclear leucocytes demonstrated a markedly reduced chemiluminescence response as well as an impaired nitroblue tetrazolium test and superoxide generation to a particulate stimulus (zymosan), while the responses to a soluble stimulus (phorbol myristate acetate) were normal. In addition, random migration und chemotactic response to zymosan-activated serum were impaired. The lymphocytes demonstrated abolished natural killer cell cytotoxicity as well as abnormal humoral immunity and a lack of antibody response to pertussis and tetanus antigens.- - - - - - - - - - ranking = 1.4keywords = antigen (Clic here for more details about this article) |
6/10. Immunologic myopathy. Linear IgG deposition and fulminant terminal episode.The histopathologic characteristics of a patient with progressive myopathy, renal failure, hemoptysis, and a fulminant terminal event demonstrated a diffuse vasculitis, type 2 fiber atrophy of muscle, and linear IgG staining of the basement membranes of skeletal muscle fibers and of glomerular capillary basement membranes. Interpretation of electron micrographs confirmed that there was thickening of muscle basement membranes. The data suggest the presence of an antibody to a basement membrane antigen shared by muscle and kidney.- - - - - - - - - - ranking = 0.2keywords = antigen (Clic here for more details about this article) |
7/10. Antenatal screening for fetal alloimmune thrombocytopenia: the results of a pilot study.Feto-maternal incompatibility for the human platelet antigen HPA-1a is an important cause of severe fetal thrombocytopenia. The incidence is 1 in 1000-2000 pregnancies, which is more common than other conditions for which screening is presently carried out. Antenatal diagnosis and management are now available, but only for subsequent siblings following diagnosis of a previously affected infant. This study describes a pilot prospective screening programme for the antenatal detection of fetomaternal alloimmune thrombocytopenia (FMAIT) due to HPA-1a incompatibility. 3473 women were typed for HPA-1a using a method designed for large-scale typing. 71 women found to be HPA-1a negative were further tested for HLA-DR52a as a risk factor for alloimmunization. All women were monitored for the development of anti-HPA-1a throughout pregnancy and a cord full blood count was taken at delivery. Two affected pregnancies were found and treated: a singleton pregnancy was treated antenatally and a twin pregnancy after delivery. The study showed that screening for FMAIT could be established within the pre-existing antenatal red cell serology programme. It was concluded that screening should be based on platelet typing and offered regardless of parity. Further stratification, combining DR52a typing and HPA-1a antibody screening, although focusing on the group of women at greater risk, may not identify all affected pregnancies. Confirmation of the diagnosis and severity of FMAIT continues to depend on fetal blood sampling during pregnancy or cord blood samples after birth.- - - - - - - - - - ranking = 0.2keywords = antigen (Clic here for more details about this article) |
8/10. Studies of immune response in a patient with selective complete C1q deficiency.Immune response in a six-year-old girl with a rare complement defect, namely selective complete C1q deficiency, was studied. Her cell-mediated immune response (delayed hypersensitivity skin tests, E, EAC rosettes, in vitro lymphocyte transformation with phytohemagglutinin), isohemagglutinin titers, serum immunoglobulin levels, antibody titers to tetanus, Epstein-Barr virus, keyhole limpet hemocyanin, pneumococcus and bacteriophage 0 x 174 were all normal. However, she had abnormal kinetics of the antibody response to bacteriophage following primary and secondary immunizations. Her antibody titers reached a peak four weeks after primary immunization and three weeks after secondary immunization, while the titers of controls peaked at two weeks and one week, respectively. In this study we could not prove the hypothesis that defective antibody response caused recurrent infections in this patient. An alternative hypothesis is the possible role of defective clearance of immune complexes in the development of severe infections. patients with selective complete C1q deficiency form immune complexes with bacterial or viral antigens, thus activating the classical complement pathway. As a result, very low C1q levels decrease even further, leading to a severe immunodeficiency and recurrent infections. Future studies in this area may help to explain the mechanism(s) responsible for infections in this rare type of complement defect.- - - - - - - - - - ranking = 0.2keywords = antigen (Clic here for more details about this article) |
9/10. Eosinophilic pustular follicular reaction: a paradigm of immune dysregulation.BACKGROUND. We encountered 10 patients whose biopsies showed an eosinophilic pustular follicular reaction, a histomorphology alleged to be pathognomonic of eosinophilic folliculitis (EF). Only seven of these patients fell within the clinical spectrum of EF. Seven patients had conditions associated with immune dysfunction, including three patients with an atopic history. Potential antigenic stimuli could be elicited in six cases. methods. Formalin-fixed biopsy specimens from all 10 patients were available for examination. hematoxylin and eosin-, alcian blue-periodic acid-Schiff (PAS)- and PAS-diastase-stained sections cut from paraffin-embedded tissue were examined by light microscopy. Immunoperoxidase preparations with antibody to IgE were performed on paraffin sections and the number of IgE-decorated cells quantitated in each case. Four patients also had biopsy material submitted in Michel's medium, on which direct immunofluorescent studies were conducted. RESULTS. IgE-coated mononuclear cells were present in patients whose lesions would logically be expected to derive from dominant type I hypersensitivity mechanisms and absent or minimal in biopsies from those patients in whom the pathogenetic basis of lesions derived principally from cell-mediated immunity. CONCLUSIONS. The eosinophilic pustular follicular reaction, while characteristic of EF, is not exclusive to that entity. It represents an expression of an excessive immediate or delayed-type hypersensitivity reaction to various auto-, epicutaneous, or ingested stimuli. A background of immune dysregulation may be contributory.- - - - - - - - - - ranking = 0.2keywords = antigen (Clic here for more details about this article) |
10/10. Necrotizing herpetic retinopathies. A spectrum of herpes virus-induced diseases determined by the immune state of the host.PURPOSE: Necrotizing herpetic retinopathies (NHR), a new spectrum of diseases induced by viruses of the herpes family (herpes simplex virus, varicella-zoster virus and cytomegalovirus), includes acute retinal necrosis (ARN) occurring in apparently immunocompetent patients and progressive outer retinal necrosis (PORN) described in severely immuno-compromised patients. Signs of impaired cellular immunity were seen in 16% of ARN patients in a review of 216 reported cases, indicating that immune dysfunction is not only at the origin of PORN but might also be at the origin of ARN. The aim of this study was to correlate clinical findings in NHR patients with their immunologic parameters. methods: charts from patients with the diagnosis of ARN or PORN seen from 1990 to 1995 were reviewed. Clinical characteristics and disease patterns were correlated with immunological parameters taking into account CD4 lymphocyte rate in AIDS patients and blood-lymphocyte subpopulation determination by flow cytometry, cutaneous delayed type hypersensitivity testing and lymphocytic proliferation rate to seven antigens in hiv-negative patients. RESULTS: During the period considered, 11 patients and 7 patients fulfilled the criteria of ARN and PORN respectively. Immune dysfunctions were identified in most patients. Mild type of ARN and classical ARN were associated with discrete immune dysfunctions, ARN with features of PORN was seen in more immunodepressed patients and classical PORN was always seen in severely immunodepressed hiv patients. CONCLUSION: Our findings suggest that NHR is a continuous spectrum of diseases induced by herpes viruses, whose clinical expression depends on the immune state of the host going from mild or classical ARN at one end in patients with non-detectable or slight immune dysfunction to PORN in severely immunodepressed patients at the other end and with intermediary forms between these extremes.- - - - - - - - - - ranking = 0.2keywords = antigen (Clic here for more details about this article) |
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