1/136. Analysis of corneal crystalline deposits in multiple myeloma.A 46-year old woman and a 59-year-old man had corneal crystals, multiple myeloma, and IgG kappa hypergammaglobulinemia. In one case, crystalline deposits were also present in the lens. In both patients there was a marked decrease in the amount of crystals during chemotherapy. The crystals within the cornea of one case were identified by light and electron microscopy in material obtained during a lamellar keratoplasty. The crystalline deposits were located only in the corneal epithelium, and their regular repeating internal arrangement was confirmed by monochromatic optical diffraction of electron micrographs of sections through them. By immunofluorescent and immunoperoxidase techniques, the crystals reacted positively for immunoglobulin and particularly IgG kappa chains.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
2/136. Crescentic glomerulonephritis in hyper IgD syndrome.The hyperimmunoglobulinemia D syndrome (HIDS) is a well-defined entity resembling familial mediterranean fever. HIDS is a systemic inflammatory disease associated with stimulation of T-cell-mediated immunity. These patients are at low risk for amyloidosis and are not known to develop nephropathy. We report a boy of Mediterranean ancestry who exhibited typical HIDS and end-stage renal failure. kidney biopsy revealed pauci-immune crescentic glomerulonephritis (cGN). We hypothesized that the glomerular involvement was secondary to the cytokine network activation observed in HIDS. Thus, cGN should be considered as part of the syndrome, and kidney biopsy should be performed early in the course of the renal disease in patients with HIDS.- - - - - - - - - - ranking = 0.44988404701176keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
3/136. Primary sjogren's syndrome in children and adolescents: proposal for diagnostic criteria.OBJECTIVE: Primary sjogren's syndrome (pSS) in childhood is a rare disease. Diagnostic criteria are available for adult patients only. In order to establish diagnostic criteria for juvenile pSS an analysis of 7 girls and one boy suffering from pSS with early onset is reported. Due to the rarity of the disease, data on patients with pSS reported in the literature are included in the proposal for modified diagnostic criteria. methods: The diagnosis of pSS was established according to the criteria for adulthood pSS, duly modified, which include clinical symptoms and laboratory immunological evaluation. RESULTS: The average age of our patients at clinical onset was 13.5 years (range: 10-17 yrs.). Clinical signs included systemic (fever, fatigue) as well as local (parotitis, vulvovaginitis, conjunctivitis) symptoms. paralysis due to hypokalemia linked to renal tubular acidosis and central nervous system (CNS) involvement was seen in one patient. Asymptomatic renal tubular acidosis was diagnosed in another 2 patients. Autoimmune hepatitis was present in 2 patients. All patients had laboratory abnormalities: hyperimmunoglobulinemia IgG, high titers of antinuclear antibodies (anti-SS-A and/or anti-SS-B) and elevated serum amylases. Sicca syndrome was never seen during childhood, although it developed later in 3 patients, after 7 to 10 years of follow-up. CONCLUSIONS: It has been stressed that the classical diagnostic criteria for adult sjogren's syndrome, especially sicca syndrome, are not applicable to a pediatric onset of the disease. On the other hand, the presence of typical laboratory abnormalities can allow the diagnosis of these patients in the early stages. Both laboratory and clinical symptoms typical for childhood are included in our proposal for diagnostic criteria applicable to juvenile pSS. life-threatening conditions such as hypokalemic paralysis, CNS involvement and hepatitis may also occur in children. Sicca syndrome tends to develop much later in pediatric patients.- - - - - - - - - - ranking = 0.44988404701176keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
4/136. Oligo-/monoclonal gammopathy and hypergammaglobulinemia in ataxia-telangiectasia. A study of 90 patients.We investigated the presence of hypergammaglobulinemia and oligo-/monoclonal gammopathy in 90 patients (from 80 families) with ataxia-telangiectasia ranging in age from 2 to 29 years. Of the 90 patients, 38.8% displayed hypergammaglobulinemia. An isolated increase in IgM was the most common finding (23.3%) followed by a simultaneous increase in IgM and IgG (8.8%), an isolated increase in IgA (3.3%), an elevated level of IgG (2.2%) and a concomitant increase in IgM and IgA (1.1%), respectively. Seven of the patients (8.1%) had oligo-/monoclonal gammopathy. The gammopathies included all major immunoglobulin isotypes. Chemotherapeutic intervention in 2 cases precipitated the emergence of new clones within a matter of weeks. Further investigation of oligo-/monoclonal gammopathies in these patients may lead to a clearer understanding of the clinical course and provide further insight into the underlying mechanisms of B-cell abnormalities in ataxia-telangiectasia.- - - - - - - - - - ranking = 6keywords = hypergammaglobulinemia (Clic here for more details about this article) |
5/136. New corneal findings in human T-cell lymphotrophic virus type 1 infection.PURPOSE: Human T-cell lymphotrophic virus type 1 is a rna retrovirus that primarily affects CD4 T-cells. Human T-cell lymphotrophic virus type 1 infection is the established cause of adult T-cell leukemia/lymphoma, an aggressive malignancy of CD4 T-cells, and two nonneoplastic conditions: human T-cell lymphotrophic virus type 1-associated myelopathy/tropical spastic paraparesis and human T-cell lymphotrophic virus type 1 uveitis. Other reported ophthalmic manifestations of human T-cell lymphotrophic virus type 1 infection include lymphomatous and leukemic infiltrates in the eye and ocular adnexa in patients with adult T-cell leukemia/lymphoma, retinal pigmentary degeneration, and neuro-ophthalmic disorders in patients with human T-cell lymphotrophic virus type 1-associated myelopathy/tropical spastic paraparesis and keratoconjunctivitis sicca, episcleritis, and sclerouveitis in asymptomatic human T-cell lymphotrophic virus type 1 carriers. This report describes the ocular findings in three Jamaican patients with human T-cell lymphotrophic virus type 1 infection and adult T-cell leukemia/lymphoma. methods: The clinical records of three patients with human T-cell lymphotrophic virus type 1 infection and adult T-cell leukemia/lymphoma examined at the National eye Institute were reviewed. Each patient had one or more complete ophthalmic evaluations. RESULTS: All three patients had corneal abnormalities, including corneal haze and central opacities with thinning; bilateral immunoprotein keratopathy; and peripheral corneal thinning, scarring, and neovascularization. All three patients had elevated serum immunoglobulin levels. CONCLUSIONS: We believe that the novel corneal findings in these patients are most likely a consequence of the hypergammaglobulinemia induced by the human T-cell lymphotrophic virus type 1 infection or the T-cell malignancy.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
6/136. Primary granulocytic sarcoma presenting with bone pain and hypergammaglobulinemia.Granulocytic sarcoma (GS), is an extramedullary tumorous aggregate of neoplastic myeloid precursor cells, most often associated with acute myeloid leukemia (AML). Primary GS occurs in patients with normal bone marrow and no history of hematological disorders. It is a rare disease, which can involve any organ and mimic other tumors. A correct initial diagnosis, which can be difficult, and early treatment with chemotherapy as for AML patients results in a higher rate of complete remission. We report a case of multifocal primary GS of the bone associated with oligoclonal hypergammaglobulinemia, successfully treated with AML-like induction chemotherapy followed by postinduction therapy with autologous peripheral stem cells transplantation. The possible significance of the associated hypergammaglobulinemia is discussed.- - - - - - - - - - ranking = 6keywords = hypergammaglobulinemia (Clic here for more details about this article) |
7/136. Tubulointerstitial nephritis and uveitis syndrome with autoantibody directed to renal tubular cells.The pathogenesis of tubulointerstitial nephritis and uveitis (TINU) syndrome remains unknown, but T cell-mediated immune response has been postulated to play a role. On the other hand, TINU syndrome is characterized by hypergammaglobulinemia and high serum immunoglobulin g (IgG) levels, suggesting an involvement of humoral immunity. We describe a case of TINU syndrome in a 13-year-old girl with multiple tubular dysfunctions including renal glucosuria, tubular proteinuria, phosphaturia, uricosuria, and concentrating and acidifying defect. IgG antibody from her serum was reactive against 125-kDa human kidney protein. Immunofluorescence study using mouse kidney revealed that the antibody was against cortical renal tubular cells. The antibody disappeared as the renal symptoms resolved. We suggest that IgG antibody may contribute to tubular dysfunction in some patients with TINU syndrome.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
8/136. Inflammatory pseudotumor of the submandibular gland: report of a case presenting with autoimmune disease-like clinical manifestations.We report a rare case of inflammatory pseudotumor arising in the submandibular gland, which presented with autoimmune disease-like clinical manifestations. A 70-year-old Japanese man developed masses in both submandibular regions. Laboratory tests revealed polyclonal hypergammaglobulinemia, high titers of antinuclear antibody, and a positive thyroid test. Histologically, the lesion was composed of multiple nodules separated by thick fibrous bands and contained a few atrophic lymphoid follicles and residual ductal structures. At higher magnification, the nodules contained numerous mature plasma cells mixed with myofibroblasts, lymphocytes, and histiocytes. Occasionally, the myofibroblasts were arranged in poorly formed fascicles and in a storiform pattern. polymerase chain reaction analysis failed to demonstrated the rearrangement of the immunoglobulin heavy-chain gene. The patient was free of disease after 72 months follow-up. Marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type arising from salivary glands occasionally showed prominent plasma cell differentiation. The present case indicates that inflammatory pseudotumor should be added to the list of different diagnoses for mucosa-associated lymphoid tissue-type lymphoma of the salivary glands.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
9/136. pathology of autoimmune myelofibrosis. A report of three cases and a review of the literature.We identified 3 patients with autoimmune myelofibrosis (AM) lacking American Rheumatism association criteria for systemic lupus erythematosus (SLE). They had 1 or 2 cytopenias and lacked serologic evidence for SLE. Autoimmune features included psoriatic arthritis and positive direct coombs test (DCT) result, DCT-positive autoimmune hemolytic anemia, and synovitis with polyclonal hypergammaglobulinemia. bone marrow biopsy specimens from each patient were evaluated by routine morphologic and immunohistochemical examination. They demonstrated marked hypercellularity (2 cases) or hypocellularity (1 case), moderate erythroid hyperplasia (all cases) with left-shifted maturation (2 cases), intrasinusoidal hematopoiesis (all cases), slightly to moderately increased megakaryocytes (2 cases), and grade 3 to 4 reticulin fibrosis (all cases). All lacked basophilia, eosinophilia, bizarre megakaryocytes, clusters of megakaryocytes, and osteosclerosis. Mild to moderate bone marrow lymphocytosis was noted in all cases. In 2 cases, increased small T cells and B cells formed nonparatrabecular, loose aggregates. AM is a clinicopathologic entity that may lack features of SLE. Loose aggregates of bone marrow T and B lymphocytes and the absence of morphologic and clinical features of myeloproliferative disease or low-grade lymphoproliferative disease are clues that distinguish AM from better known causes of bone marrow fibrosis.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
10/136. Defective neutrophil chemotaxis with variant ichthyosis, hyperimmunoglobulinemia E, and recurrent infections.Host defense mechanisms were evaluated in a 4-1/2-year-old boy with recurrent pyogenic infections and a unique hyperkeratotic skin disorder. The patient's neutrophils were consistently defective in chemotactic responsiveness but had normal NBT reduction, glucose oxidation, and iodination. serum concentrations of IgE were markedly elevated and the secondary antibody response was abnormal. No T-cell dysfunction was detected. These findings suggest a relationship between this patient and patients with other syndromes associated with recurrent infections, cutaneous disease, defective chemotaxis, immunodeficiency, and hyperimmunoglobulinemia E.- - - - - - - - - - ranking = 2.2494202350588keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
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