1/73. Selective antibody deficiency to bacterial polysaccharide antigens in patients with netherton syndrome.Three patients with netherton syndrome, recurrent sinopulmonary infections, and humoral immune deficiency are described. Although quantitative serum immunoglobulin levels were generally normal, two patients had selective antibody deficiency to bacterial polysaccharide antigens, one associated with IgA-IgG-2 deficiency. A third patient had an antibody deficiency to protein antigens. This is the first report, to our knowledge, that describes antibody deficiency in patients with netherton syndrome. This finding demonstrates the importance of evaluating functional antibody responses to both protein and bacterial polysaccharide antigens and not relying on IgG subclass determination.- - - - - - - - - - ranking = 1keywords = q (Clic here for more details about this article) |
2/73. The transfusion needs of an autologous bone marrow transplant patient with iga deficiency.BACKGROUND: Transfusion management of the patient who is undergoing a marrow or peripheral blood stem and progenitor cell transplantation is often challenging. The situation is further complicated when the patient is IgA deficient with circulating anti-IgA. CASE REPORT: This report describes an approach to transfusion therapy primarily using red cells washed by automated techniques and cryopreserved autologous plateletpheresis components. Additional platelet support was provided with manually washed allogeneic plateletpheresis components. Autologous fresh-frozen plasma was collected concurrently, and IgA-deficient allogeneic units were ordered and kept in storage, but they were not needed during transplantation. The patient experienced no transfusion sequelae as a result of the iga deficiency. CONCLUSION: With this approach, the transfusion needs of an IgA-deficient patient were adequately met during bone marrow transplantation.- - - - - - - - - - ranking = 3keywords = q (Clic here for more details about this article) |
3/73. Reversible total iga deficiency associated with phenytoin treatment.A 55-y-old male developed long-standing upper respiratory symptoms during phenytoin treatment of epileptic fits. Complete lack of serum IgA was noted repeatedly. Following replacement of phenytoin, normal IgA levels were found and a slow improvement in respiratory symptoms occurred. Immunoglobulin deficiency, in particular low levels of IgA, are not uncommon among phenytoin treated subjects. The occurrence of frequent respiratory infections in such patients justifies the investigation of immunoglobulin levels.- - - - - - - - - - ranking = 1keywords = q (Clic here for more details about this article) |
4/73. nephrotic syndrome in a patient with iga deficiency-associated mesangioproliferative glomerulonephritis.A case of mesangioproliferative glomerulonephritis in a 55-year-old woman with selective iga deficiency and serum antinuclear antibodies who presented with nephrotic syndrome is described. The patient did not have clinical or laboratory features of systemic lupus erythematosus (SLE) other than antinuclear antibodies. histology of the patient's renal biopsy revealed a mesangioproliferative glomerulonephritis and direct immunofluorescence showed that paramesangial deposits contained predominant IgM with lesser IgG, C3 and C1q. These findings are identical to those previously described in a form of glomerulonephritis associated with iga deficiency and would be atypical for lupus nephritis. glomerulonephritis is not a well recognized complication of iga deficiency, though it has been rarely reported in the literature. This case provides further evidence that iga deficiency is associated with a unique immune complex-mediated glomerulopathy with characteristic immunopathological and ultrastructural features. It is the first reported case to present with nephrotic syndrome.- - - - - - - - - - ranking = 2keywords = q (Clic here for more details about this article) |
5/73. Evolution of iga deficiency to IgG subclass deficiency and common variable immunodeficiency.FIRST REPORT: male child with repeated pulmonary infections from the age of 4 months. He was diagnosed as iga deficiency (undetectable IgA levels) at the age of 3 years, when he presented repeated bouts of pneumonia and tonsillitis. Several immunologic evaluations were made between the ages of 4 months and 8 years. At 8 years and 9 months, the diagnosis of iga deficiency was confirmed, and associated IgG2 and IgG4 deficiency (29.0 mg/dl y 0.01 mg/dl) with normal total IgG serum level was found. With the administration of intravenous gammaglobulin, the lung infections remitted and the subsequent clinical course has been uneventful up to now. SECOND REPORT: a boy with repeated infections since the age of 2 months. iga deficiency was diagnosed at 1 year 7 months (undetectable serum IgA levels). At age 51/2 years, his clinical course worsened and more serious infections appeared. A new immunologic study revealed iga deficiency associated with CD4 cell deficiency (432 cells/mm3) and normal CD3, CD19, and CD8 levels. Despite intensive antibiotic treatment and care, the child died. The findings suggest an association of iga deficiency and common variable immunodeficiency.- - - - - - - - - - ranking = 1keywords = q (Clic here for more details about this article) |
6/73. sarcoidosis and common variable immunodeficiency. A case of a malignant course of sarcoidosis in conjunction with severe impairment of the cellular and humoral immune system.The occurrence of sarcoidosis in combination with common variable immunodeficiency (CVID) has been described in a small number of patients. In these patients, sarcoidosis consisted of lymphadenopathy, mild to moderate pulmonary involvement and hepatosplenomegaly. However, severe and rapidly progressive pulmonary fibrosis in combination with a severe defect of the cellular and humoral immune system has not been described yet. In our patient, defects of the T and B cell system resulted in severe immunodeficiency. The defect of the humoral immune system was characterized by the impairment of specific antibody production in vivo. In addition, hypogammaglobulinemia with missing IgA and IgE along with a marked defect in IgM and IgG production was noted. There was a progressively reduced lymphocyte proliferation in response to T cell mitogens, while proliferation after specific IL-2 stimulation was normal. A Th1 lymphocyte-subset-like profile might thus play a role in the pathogenesis and might form the connecting link between sarcoidosis and CVID. This is the report of a so far new and unique combination of severe immunodeficiency and sarcoidosis also associated with a congenital dysmorphia consisting of a palatal cleft. The findings of the 40 patients with CVID and sarcoidosis reported so far are discussed in order to point out the typical features of patients with this uncommon syndrome.- - - - - - - - - - ranking = 1keywords = q (Clic here for more details about this article) |
7/73. Juvenile idiopathic polyarticular arthritis and iga deficiency in the 22q11 deletion syndrome.Five patients with the 22q11 deletion syndrome (velocardiofacial syndrome) developed chronic inflammatory polyarticular arthritis. These new cases add to 8 previously reported and confirm the association. The arthritis in all cases was moderate to severe, but at least partially responsive to methotrexate and/or corticosteroids, and was clinically indistinguishable from juvenile idiopathic arthritis (JIA). Analysis of the total 13 patients indicates that 2 are rheumatoid factor positive, 6 are antinuclear antibody positive, 5 have subtle T cell deficiencies, and 6 have hypergammaglobulinemia. Of particular interest is the occurrence of iga deficiency in 4 patients, including 2 from our own series. Although iga deficiency is seen in both JIA (2-4%) and 22q11 deletion syndrome (2-4%), the prevalence of low IgA in this series (31%) is much greater than expected. This phenomenon and the true association of inflammatory arthritis and a chromosome deletion disorder provides further evidence of important genetic factors in the pathogenesis of JIA.- - - - - - - - - - ranking = 131841.74308106keywords = deletion syndrome, deletion, q (Clic here for more details about this article) |
8/73. Sonographic detection of multiple brain abscesses in a newborn with iga deficiency.We report the case of a neonate with selective iga deficiency and multiple brain abscesses diagnosed with sonography. brain sonography revealed multiple abscesses in the left hemisphere; the abscesses ranged from 10 to 20 mm. Cultures obtained from the cerebrospinal fluid and blood were positive for proteus mirabilis. The neonate responded promptly to broad-spectrum antibiotic therapy and had no neurologic sequelae. Because iga deficiency is associated with infections, we believe it was a predisposing factor for the brain abscesses.- - - - - - - - - - ranking = 1keywords = q (Clic here for more details about this article) |
9/73. Anti-D immunoglobulin treatment for thrombocytopenia associated with primary antibody deficiency.AIMS: To review our experience of anti-D immunoglobulin for immune thrombocytopenia (ITP) in patients with primary antibody deficiency. methods/patients: A retrospective case notes review of four Rhesus positive patients with ITP and primary antibody deficiency, treated with anti-D. patients were refractory to steroids and high dose intravenous immunoglobulin (IVIG). Two patients were previously splenectomised. RESULTS: All patients responded to anti-D immunoglobulin. Improved platelet counts were sustained for at least three months. Side effects included a fall in haemoglobin in all cases; one patient required red blood cell transfusion. Two patients had transient neutropenia (< 1 x 10(9)/litre). CONCLUSION: Anti-D immunoglobulin may be an effective treatment for antibody deficiency associated thrombocytopenia, even after splenectomy. Anti-D immunoglobulin may have considerable clinical advantages in this group of patients, where treatments resulting in further immunosuppression are relatively contraindicated.- - - - - - - - - - ranking = 1keywords = q (Clic here for more details about this article) |
10/73. plasma cell gingivitis.plasma cell gingivitis (PCG), an infrequent benign inflammatory condition of unknown etiology, is a type of plasma cell orificial mucositis, which includes a wide spectrum of conditions. We present the case of a 13-year-old girl who had PCG with an erythematous congestive plaque on the anterior maxillary gingiva for 4 years. Occasionally, the lesion became increasingly swollen and painful and bled. Results of a histopathologic examination showed dense plasmacytic infiltrate in the dermis, affecting the dermoepidermal border, with immunohistochemical positivity in the K and A light chains and vascular proliferation. "Lozenge" keratinocytes, "watery" spongiosis, and exocytosis were seen in the epidermis. Laboratory analysis showed notably low levels of both serum IgA and secretory IgA. We consider whether secretory IgA at low levels has an important etiopathogenic role favoring the development of localized subclinical repetitive infections that could lead to chronic PCG.- - - - - - - - - - ranking = 2keywords = q (Clic here for more details about this article) |
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