21/87. Polyclonal IgG4 hypergammaglobulinemia associated with plasmacytic lymphadenopathy, anemia and nephropathy.Marked polyclonal immunoglobulin (Ig)G4 hypergammaglobulinemia has exceptionally been reported. Here we report on two Algerian patients who presented a syndrome characterized by anemia, plasmacytic lymphadenopathy, renal manifestations, and a marked polyclonal IgG4 hypergammaglobulinemia leading to a hyperviscosity syndrome in one case. The IgG4-expressing cell percentage was significantly increased in the peripheral blood lymphocytes collected from the two patients upon diagnosis. Moreover, in contrast with normal sera, both patients' sera significantly increased the percentage of IgG4-expressing cells when incubated with CD40-stimulated normal B lymphocytes. Similar effects were obtained with the culture supernatants of the patients' activated T cells. Anti-interleukin (IL) 4 and/or anti-IL-13 antibodies were unable to antagonize the IgG4 production. IL-4 and IL-13 serum concentrations were found to be normal in the two patients. The increased IgG4 production was found to be mediated by soluble factor(s), most probably secreted by activated T cells, which did not require the signal transducer and activator of transcription 6 signaling pathway.- - - - - - - - - - ranking = 1keywords = peripheral (Clic here for more details about this article) |
22/87. Chronic inflammatory demyelinating polyneuropathy associated with idiopathic hemochromatosis.We describe a 50-year-old woman who developed chronic inflammatory demyelinating polyneuropathy (CIDP) one year after onset of hemochromatosis. Electrodiagnostic studies showed evidence of multifocal demyelination. Marked hypergammaglobulinemia with positive anti-nuclear and anti-dna antibodies was found. Corticosteroid treatment resulted in a significant lessening of neurological symptoms. This is the first case of CIDP with hemochromatosis. The association may be coincidental, but the altered immune system by hemochromatosis was possibly related to the development of CIDP in this patient.- - - - - - - - - - ranking = 1.3209538423687keywords = neuropathy (Clic here for more details about this article) |
23/87. Polyclonal plasma cell proliferation with marked hypergammaglobulinemia and multiple autoantibodies.A 77-yr-old man presented with marked peripheral blood and bone marrow plasmacytosis, marked hypergammaglobulinemia, and multiple autoantibodies. serum protein immunofixation and immunophenotyping of bone marrow plasma cells by flow cytometry and immunohistochemistry disclosed polyclonal proliferation of plasma cells at various stages of differentiation. The presence of multiple autoantibodies in the patient's serum suggests that an autoimmune disease underlies the polyclonal proliferation of plasma cells.- - - - - - - - - - ranking = 1keywords = peripheral (Clic here for more details about this article) |
24/87. immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma in a child.We report the case of a 14-year-old Japanese boy with peripheral T-cell malignant lymphoma, showing progression from immunoblastic lymphadenopathy (IBL) to overt malignant lymphoma. He suffered recurrent fever, generalized lymphadenopathy, hepatosplenomegaly and maculopapular exanthema. leukocytosis with eosinophilia and polyclonal hypergammaglobulinemia were observed during the aggressive course of the disease. In the early phase, human immunoglobulin and steroids improved the symptoms but did not induce complete remission, and the patient died one year after the onset of the illness. Four biopsies of lymph nodes revealed progression from IBL to CD4 positive T-cell lymphoma through IBL-like T-cell lymphoma. Though IBL-like T-cell lymphoma is defined as IBL with neoplastic features and overt T-cell malignant lymphoma progressed from IBL-like T-cell lymphoma is excluded from the definition, it may be preferable that such malignant lymphoma as our case should also be included in IBL-like T-cell lymphoma.- - - - - - - - - - ranking = 1keywords = peripheral (Clic here for more details about this article) |
25/87. T helper-cell leukemia/lymphoma: presentation as an acute immune-mediated illness.A 36-year-old man presented with an acute immune-mediated illness characterized by leukocytoclastic vasculitis and polyarthritis. Evaluation of the synovial fluid, bone marrow, and peripheral blood revealed large numbers of abnormal lymphoid cells labeling a 4B4-positive, CD4-positive, IL-2 receptor-negative, helper T cells. hypergammaglobulinemia, immune complexes, high levels of serum IL-2 receptors, serum antibodies against foreign alloantigens, and specific cytolysis of the patient's leukemic cells by his normal CD8 T lymphocytes suggest an interaction of the malignant cells and his normal immune cells. Thus, some of the rheumatologic symptoms leading to the diagnosis of leukemia appear to reflect an immunoregulatory imbalance manifested by B-cell hyperactivity, likely induced by the malignant helper T cells, and attempted regulation of his malignant T cells by normal lymphocytes.- - - - - - - - - - ranking = 1keywords = peripheral (Clic here for more details about this article) |
26/87. plasma exchange: a treatment for neuropathy associated with IgG-kappa gammopathy.A patient with a peripheral neuropathy associated with an IgG-kappa gammopathy is reported who was treated with plasma exchange as the only therapeutic modality and showed dramatic clinical improvement, which fluctuated based on the frequency of plasma exchange. Therapeutic plasma exchange is recommended as one form of possible treatment for neuropathies resulting from IgG gammopathies. The literature is reviewed.- - - - - - - - - - ranking = 4.4624939912272keywords = peripheral neuropathy, peripheral, neuropathy (Clic here for more details about this article) |
27/87. Peripheral neuropathy in monoclonal gammapathy with cryoglobulinemia and arteritis.We report a patient with IgM gammapathy, cryoglobulinemia, Raynaud's phenomena, purpura hyperglobulinemica of the legs and polyneuropathy. Endoneural vasculitis with infiltrations of eosinophilic and neutrophilic granulocytes and an extensive loss or wallerian degeneration of myelinated nerve fibers were seen on histopathologic examination of a sural nerve biopsy specimen. The microscopic picture differed somewhat from that observed previously in cryoglobulinemic vasculitis. Although vasculitis is most often believed to represent an immunologically mediated lesion, we propose an alternate explanation, namely, that the disease manifestations in the present case were secondary to cold-induced effects of the cryoglobulin on the microcirculation.- - - - - - - - - - ranking = 1.3311736295561keywords = neuropathy, nerve (Clic here for more details about this article) |
28/87. A case of histiocytosis X associated with panhypopituitarism and hyperimmunoglobulinemia G and E.A 43 year old man with diabetes insipidus who showed panhypopituitarism and marked hypergammaglobulinemia due to histiocytosis X is reported. His low basal plasma adrenocorticotropin (ACTH) and growth hormone (GH) failed to respond to insulin-induced hypoglycemia. His basal serum thyroid hormone level was below normal and normal basal plasma thyrotropin (TSH) showed a delayed response with normal peak value to TSH-releasing hormone (TRH). Normal basal plasma pituitary gonadotropin also showed a delayed response with normal peak value to luteinizing hormone-releasing hormone (LH-RH). Suppression of plasma prolactin (PRL) by levodopa (l-dopa) was impaired and elevation of basal plasma PRL was noted at the second admission. These results, combined with diabetes insipidus, suggested that the panhypopituitarism in these patients was hypothalamic in origin. The polyclonal hypergammaglobulinemia was characterized by elevated serum IgG and IgE levels which returned to normal after corticosteroid treatment with concomitant clinical improvement. Elevated serum IgE levels, tissue and peripheral eosinophilia, and the effectiveness of corticosteroid therapy support the hypothesis that some allergic mechanism may be involved in the pathogenesis of this disease.- - - - - - - - - - ranking = 1keywords = peripheral (Clic here for more details about this article) |
29/87. synovitis in angioimmunoblastic lymphadenopathy with dysproteinemia simulating rheumatoid arthritis.We describe a patient with angioimmunoblastic lymphadenopathy with dysproteinemia who developed a symmetric, rheumatoid-like, peripheral polyarthritis. Radiographs of the involved joints revealed soft tissue swelling without erosions or cartilage loss. rheumatoid factor and fluorescent antinuclear antibodies were negative, and c-reactive protein and erythrocyte sedimentation rate were normal. synovial fluid analysis showed an inflammatory effusion (white blood cell count of 3,500/mm3, with 76% polymorphonuclear leukocytes). A closed synovial biopsy of the wrist revealed a mononuclear infiltrate consistent with angioimmunoblastic lymphadenopathy with dysproteinemia. Monthly parenteral chemotherapy treatment with high-dose methyl-prednisolone and cyclophosphamide resulted in remission of all manifestations of disease, including arthritis.- - - - - - - - - - ranking = 1keywords = peripheral (Clic here for more details about this article) |
30/87. Selective deficiency in IL-2 production and refractoriness to extrinsic IL-2 in immunodeficiency with hyper-IgM.Various lymphokines are inducible by the stimulation of T-cell mitogens, phytohemagglutinin, and concanavalin a. A 32-year-old female with an atypical type of immunodeficiency with hyper-IgM was evaluated for possible defects in the production of several immunoregulatory lymphokines. Although the mitogens appeared to bind effectively to the specific surface receptors of patient peripheral blood lymphocyte (PBL), the proliferative responses were significantly decreased. The culture supernatant of patient PBL stimulated by the mitogens contained only a trace amount of interleukin 2 (IL-2) activity. Addition of recombinant IL-2 to the cultures concomitantly with the mitogens could not restore the decreased responses of patient PBL. Tac antigen expression of patient PBL induced by the mitogens was moderately impaired. These data suggest that there is a defect in both IL-2-producing and IL-2-responding cells. In contrast, the culture supernatant of mitogen-stimulated patient PBL contained B-cell growth and differentiation factors as well as interferon-gamma activities equal to those of the control. These results suggest that there are independent regulatory pathways for the production of IL-2 and other T-cell-derived lymphokines.- - - - - - - - - - ranking = 1keywords = peripheral (Clic here for more details about this article) |
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