1/17. Selective antipolysaccharide antibody deficiency associated with peripheral blood CD5 B-cell predominance.BACKGROUND: Primary humoral deficiencies vary from complete absence of B cells and/or serum immunoglobulin to lacunar deficits involving specific antibody responses to polysaccharides. OBJECTIVES: We compared the B-cell CD5 expression in patients with selective antipolysaccharide antibody deficiencies (SPADs), common variable immunodeficiency (CVID), and IgG subclass deficiency and in normal control subjects. methods: Five patient populations were evaluated: (1) patients with severe SPAD (no protective serologic postvaccine response to any of 12 polysaccharide antigens tested); (2) patients with intermediate SPAD (diminished response to polysaccharide antigens and adequate response to 1 to 3 of 12 serotypes tested); (3) patients with IgG subclass deficiency; (4) patients with CVID; and (5) age-matched control subjects. blood was collected from all patients and evaluated by using flow cytometry. Results were compared by using the Student t test. RESULTS: patients with severe SPAD deficiencies had a marked predominance of CD5 B cells in the peripheral blood (93% to 97% of total B cells, n = 2). The intermediate SPAD group had a mean CD5 B-cell percentage that was significantly higher than that of the age-matched control group (87. 4%, n = 7, vs 52.5%, n = 20; P =.007). patients with CVID and IgG subclass deficiency had mean CD5 B-cell percentages that were similar to those of the age-matched control subjects. CONCLUSIONS: Our studies demonstrate that patients with SPAD had a markedly increased percentage of CD5 B cells in the peripheral blood as compared with age-matched control subjects and patients with other humoral deficiencies. This observation suggests that an association may be present between CD5 B-cell predominance and SPAD.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/17. Polymorphic light eruption occurring in common variable hypogammaglobulinaemia, and resolving with intravenous immunoglobulin therapy.A 55-year-old woman with a past history of lower respiratory tract infections presented with a photosensitive eruption. Polymorphic light eruption (PLE) was diagnosed on the basis of the temporal relationship to sun exposure and the diagnosis was supported by positive monochromator irradiation tests in the ultraviolet A wavelength spectrum. Investigation of the patient's immune status identified low levels of all immunoglobulin (Ig) subtypes consistent with common variable hypogammaglobulinaemia. Intravenous Ig replacement therapy, instituted to minimize risks from bacterial infections, was commenced and over the ensuing months resulted in a complete resolution of the PLE. PLE is considered to represent a type IV hypersensitivity reaction directed against a cutaneous autoantigen induced by exposure to ultraviolet light. In PLE, nonspecific immunomodulatory mechanisms of intravenous Ig may be active, such as a reduction in the synthesis of cytokines and a blockage of the IgG Fc receptors on macrophages.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
3/17. common variable immunodeficiency with mosaic trisomy 8: report of one case.This case study reported a 17-year-old female of common variable immunodeficiency (CVID) associated with bronchiectasis, pernicious anemia and mosaic trisomy 8. Clinically this patient presented with recurrent sinopulmonary infections, intractable diarrhea, macrocytic anemia, and primary amenorrhea. Immunological tests showed pan-hypogammaglobulinemia and a decrease of peripheral blood B cells (4%) and CD4 cells (25%). Lymphoproliferative responses to mitogen (PHA) and specific antigen (BCG) were profoundly impaired in the patient in comparison to those in control. Production of interleukin 4 (IL-4) and gamma interferon (IFN-gamma) in the in vitro lymphoproliferation was also profoundly depressed. Pernicious anemia demonstrated by larger MCV (112.9 fl) and hyper-segmental granulocytes on peripheral blood smear responded to parental administration of vitamin B12. Interestingly, she had a mosaic trisomy 8 in peripheral blood mononuclear cells but normal 46XX karyotype in the bone marrow cells. To our knowledge, this is the first case of CVID associated with mosaic trisomy 8 reported in the literature. As the case exemplifies, CVID should be considered when the physicians evaluate the patient presenting with recurrent sinopulmonary infections, diarrhea, malnutrition, and pernicious anemia. It requires further study to explore whether the genes in the chromosome 8 are linked to CVID.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
4/17. common variable immunodeficiency, insulin-dependent diabetes mellitus and celiac disease.common variable immunodeficiency is a disorder characterised by hypogammaglobulinemia with b-lymphocytes in peripheral blood and repeated infections. We report a child with a diagnosis of diabetes mellitus and celiac disease during lactation, and in whom common variable immunodeficiency was diagnosed at the age of 5. During evolution of the disease he presented multiple respiratory infections in spite of substitution therapy with gamma globulins. He presented pulmonary fibrosis with a pulmonary volume reduced, and a spirometric restrictive patron. Immunologically, he presents reduction in CD4 lymphoid population. He expresses the alleles DQ2 A1 0501 and B1 which are strongly associated with susceptibility to insulin-dependent diabetes mellitus and celiac disease, but don't express antigens HLA class II DR3 and DR4 that are more frequent in these entities. The main disease and all the complications had affected his curve pondostatural.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
5/17. Lymphomas of mucosal-associated lymphoid tissue in common variable immunodeficiency.common variable immunodeficiency (CVID) is a primary immunodeficiency disease characterized by low serum immunoglobulins IgG, IgA, and usually IgM. The central immune deficiency is impaired secretion of immunoglobulins and lack of antibody production; however, T cell dysfunction and a variety of inflammatory complications suggest global immune dysregulation. A number of reports have documented the association of primary immunodeficiency diseases with the development of non-Hodgkin's lymphoma (NHL). In CVID, the risk has been estimated to lie between 1.4% and 7%. As for NHL arising in other immunodeficiency states, the lymphomas in CVID are extranodal and are usually B cell in type. Of 22 B cell lymphomas that have appeared over a period of 25 years in a cohort of subjects with CVID, five lymphomas, appearing in more recently studied subjects, that arose in mucosal sites would be classified as mucosa-associated lymphoid tissue (MALT) lymphomas. MALT lymphomas are low-grade B cell lymphomas that result from a proliferation of neoplastic marginal-zone related cells of lymphoid tissue and tend to occur in organs that have acquired lymphoid tissue due to long-term infectious or autoimmune stimulation. Lymphomas of this kind have not been described in patients with congenital immunodeficiency, although chronic mucosal antigen stimulation is an integral part of these immune deficiency states.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
6/17. Antibody response in common variable immunodeficiency.BACKGROUND: common variable immunodeficiency (CVID) describes a heterogeneous group of immunologic disorders of unknown etiology. It is characterized by low levels of serum immunoglobulin (Ig) and impaired antibody response. OBJECTIVE: To describe antibody response and the kinetics of IgG decline in patients identified with CVID. methods: Clinical and immunologic observations of four patients identified with CVID were obtained by chart review. RESULTS: Antibody response to polysaccharide antigens in patients identified with CVID is lost earlier than the antibody response to protein antigens, which may be preserved even in the face of profound hypogammaglobulinemia. In three patients who were followed prospectively, the Ig loss was progressive. CONCLUSIONS: Absence of antibody response to polysaccharide antigens may be a universal finding in patients with CVID, whereas preservation of T cell-dependent protein antibody response may be seen.- - - - - - - - - - ranking = 1.5keywords = antigen (Clic here for more details about this article) |
7/17. Acute respiratory failure in a patient with sarcoidosis and immunodeficiency--an unusual presentation and a complicated course.Pulmonary sarcoidosis is usually a chronic, insidious disease resulting from granuloma formation in the lung parenchyma. The epithelioid non-caseating granulomata of sarcoidosis are the result of a T-helper1-mediated immune reaction to an unknown self or foreign antigen. We describe the case of a patient with sarcoidosis and a coexistent common variable immunodeficiency who presented with rapidly progressive respiratory failure. This unusual presentation was followed by a complicated course with recurrent pneumoccocal infections, which could be explained by the coexistence of common variable immunodeficiency. physicians should be alert to the possibility of sarcoidosis even when the clinical presentation is of acute respiratory distress syndrome (ARDS) since early treatment with steroids can be lifesaving. The detection of accompanying hypogammaglobulinemia is also crucial, as treatment with intravenous immunoglobulins (IVIG) together with steroids can improve the patient's outcome.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
8/17. Polyglandular autoimmune syndrome type III accompanied by common variable immunodeficiency.We identified polyglandular autoimmune (PGA) syndrome type III in a 24-year-old nurse with common variable immunodeficiency (CVID). An immune-mediated disorder, membranoproliferative glomerulonephritis, was diagnosed when she was 15 years old. Clinical examination and laboratory findings revealed a PGA syndrome due to the presence of hypergonadotropic hypogonadism, insufficient growth hormone response and thyroid autoimmunity. The patient had neither adrenal disease nor hypoparathyroidism. Therefore we concluded that this patient has PGA syndrome type III. This is an interesting case, because we could not find any previous report of such coexistence between PGA type III and CVID in a medline search. Coexistence of these two entities may be a result of autoimmunity and the association of both conditions with human leukocyte antigen.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
9/17. Partial response to anti-CD20 monoclonal antibody treatment of severe immune thrombocytopenic purpura in a patient with common variable immunodeficiency.Immune thrombocytopenic purpura (ITP), alone or in combination with autoimmune hemolytic anemia (Evans syndrome) and/or autoimmune neutropenia, is frequent in patients with common variable immunodeficiency (CVID). A 34-year-old man with CVID had long-standing unresponsive ITP. The patient had a 9-year history of CVID on substitutive therapy with intravenous immunoglobulin (IVIG). The clinical course of CVID was complicated with refractory fistulizing inflammatory bowel disease, nodular regenerative hyperplasia of the liver, splenomegaly, severe portal hypertension, and hypercatabolism of IgG. ITP was refractory to medical therapy, including different combinations of corticosteroids, high-dose IVIG, azathioprine, and vincristine. splenectomy was not performed because of severe portal hypertension. He received a total five doses of rituximab, a monoclonal antibody directed against CD20 antigen, at a dose of 375 mg/m(2). After an initially slow response, his platelet count increased to more than 50,000/microL by the fourth week of infusion. Therapy was well tolerated, and B lymphocytes were effectively depleted from the peripheral blood. The patient was completely tapered off glucocorticoids and maintained platelets at above 40,000/microL. The patient has not taken immunosuppressive agents for 11 months. Early treatment with rituximab might be an option for patients with CVID and ITP that do not respond to other treatments or for patients for whom a splenectomy is contraindicated.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
10/17. Successful treatment with cyclosporine A of HCV-driven chronic liver disease mimicking autoimmune hepatitis in a patient with common variable immunodeficiency.common variable immunodeficiency (CVID) is the commonest primary immunodeficiency disease characterized by defective antibody production and various degrees of T cell numbers abnormality or impaired proliferation to mitogens. Clinical features include recurrent bacterial sinopulmonary and gastrointestinal infections. autoimmunity is very common in CVID, occurring in approximately 25% of the patients particularly with autoimmune thrombocytopenia, hemolytic anemia, inflammatory bowel disease, and rheumatoid arthritis. Persistent antigen stimulation, secondary to a defective eradication of pathogens followed by a compensatory exaggerated chronic inflammatory response, is the primary cause leading to autoimmunity. Here we describe a girl with CVID in whom a chronic liver disease mimicking autoimmune hepatitis developed after hepatitis c virus infection. The immunosuppressive treatment with cyclosporine A proved effective in reversing liver disease.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
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