1/9. 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 = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
2/9. Angio-immunoblastic lymphadenopathy. diagnosis and clinical course.The clinical and pathologic findings in 24 patients with "angio-immunoblastic lymphadenopathy with dysproteinemia" (AILD) are presented. The patients' ages ranged from 44 to 80 years, with a median age of 68 years. The disease has an acute onset. In many respects, the clinical presentation is suggestive of malignant lymphoma. Generalized lymphadenopathy was always present. hepatomegaly was found in 20 patients, splenomegaly in 17, constitutional symptoms in 20 and skin rashes in nine. Twenty patients had anemia, with positive Coombs' test in eight of 14 tested. Polyclonal hypergammaglobulinemia was found in 17 of 22 patients. Two patterns of evolution were recognizable: (1) long survival (24 to 67 months) without treatment or after the administration of intensive combination chemotherapy; and (2) rapid progression (one to 19 months) regardless of the treatment given. Sixteen patients died; postmortem examination in 10 cases showed the cause of death to be attributable to severe infection in eight patients, to renal disease in one and to cardiovascular disease in one. No evidence of malignant lymphoma was seen in any of these autopsies. Histologically, the disease is systemic, with specific lesions in the lymph nodes. The spleen, liver, bone marrow, skin and lung are also involved, but the changes are less characteristic than in the lymph nodes. In the patients in whom sequential biopsies were performed, a trend toward restoration of the nodal architecture was observed. AILD is a clinical-pathologic entity in a spectrum of yet to be defined immune reactions. The clinical, laboratory and pathologic manifestations of AILD are consistent with an autoimmune disorder, in which a deficiency of the T-cell regulatory functions probably predisposes to an abnormal proliferative and autoaggressive reaction of the B-cell system. Surgical staging procedures do not appear to be indicated. Intensive cytotoxic treatment may be hazardous in some patients, precipitating their death, but long survival after such therapy has been observed in others. Supportive therapy and small doses of steroids appear to be a safer therapeutic approach.- - - - - - - - - - ranking = 0.16666666666667keywords = hypergammaglobulinemia (Clic here for more details about this article) |
3/9. Monoclonal IgA gammopathy due to maternal B cells in an infant with severe combined immunodeficiency (SCID) prior to hematopoietic stem cell transplantation.Monoclonal gammopathies result in hypergammaglobulinemia due to uncontrolled immunoglobulin production by a B-cell clonal population. They have been reported in patients with severe combined immune deficiency (SCID) after hematopoietic cell transplant, and also in normal children and in adults over 50 years of age. Generally they are benign, but malignant transformation does occur. The authors report an unusual case of a monoclonal IgA gammopathy occurring in an infant with SCID, prior to transplantation, due to engraftment of maternal B cells. Transplacental passage of maternal cells may occur more frequently than previously estimated. Persistence of activated B cells occurred even after treatment with rituximab and could pose a risk for the development of malignancies such as multiple myeloma.- - - - - - - - - - ranking = 0.16666666666667keywords = hypergammaglobulinemia (Clic here for more details about this article) |
4/9. IBL-like T cell lymphoma expressing monoclonal gammopathy (macroglobulinemia) in the serum.A case of IBL-like T cell lymphoma with serum monoclonal gammopathy was reported. A 58-year-old woman, who had suffered from heart failure, was admitted because of asthma attack, fever and lymphadenopathy. Leucopenia with a small amount of atypical lymphocytes was detected. serum analysis showed monoclonal elevation of IgM-kappa (M-protein) and hyperviscosity. Urinary Bence-Jones protein was detected. Lymph node biopsy revealed the disappearance of normal structure and proliferation of T cells with pale cells which characterized IBL-like T cell lymphoma. Immunocytochemistry revealed the pale cells to bear T cell markers (MT-1, CD 5, CD 8 or CD 4) and IgM-positive cell distribution. Tonsilar biopsy showed the infiltration of atypical lymphoids and pale cells. bone marrow biopsy showed moderate lymphoplasmacytoid proliferation with lymph follicles. Clinical data and serum analysis suggested macroglobulinemia. Additional lymph node biopsy was performed and revealed IBL-like T cell lymphoma. IBL-like T cell lymphoma is characterized by polyclonal hypergammaglobulinemia. The present case probably occurred initially as IBL-like T cell lymphoma and lymphoplasmacytoid cell proliferation might have followed due to an excess of CD 4 cells.- - - - - - - - - - ranking = 0.16666666666667keywords = hypergammaglobulinemia (Clic here for more details about this article) |
5/9. immunoblastic lymphadenopathy with purpura and cryoglobulinemia.immunoblastic lymphadenopathy is a recently described lymphoproliferative disorder, presumably of B-cell origin. It is characterized by regional or generalized lymphadenopathy, usually associated with hypergammaglobulinemia or dysproteinemia. Other findings may be hepatosplenomegaly, dermatitis, fever, malaise, weight loss, and various altered immunologic reactions. Histologically, the involved lymph nodes show immunoblast, plasmacytoid, and plasma cell proliferation. This may be extranodal as well. The case reported here is one of the few followed up prospectively. The patient's purpuric eruption was an apparent manifestation of a type II mixed cryoglobulinemia. Differing from what has usually been reported, we noted hypogammaglobulinemia and findings in part of altered cell-mediated immunity. Despite leukopenia and anemia there were no infectious episodes. Although a satisfactory treatment regimen has not been established, there was beneficial response to prednisone and short courses of melphalan.- - - - - - - - - - ranking = 0.16666666666667keywords = hypergammaglobulinemia (Clic here for more details about this article) |
6/9. Monoclonal gammopathy in hereditary spherocytosis: a possible pathogenetic relation.Two cases of monoclonal gammopathy in patients with hereditary spherocytosis led us to consider the possible pathogenetic relation between these two disorders. Twelve adult patients with hereditary spherocytosis had significant hypergammaglobulinemia in comparison to normal subjects. Retrospective analysis of previous illness in 140 patients with multiple myeloma showed a significant association between IgA myeloma and previous gallbladder disease. We propose that the chronic reticuloendothelial stimulation due to extravascular hemolysis, possibly potentiated by the inflammation associated with cholelithiasis and cholecystitis, may foster neoplastic transformation of immunocytes in patients with hereditary spherocytosis, ultimately leading to the development of monoclonal gammopathy.- - - - - - - - - - ranking = 0.16666666666667keywords = hypergammaglobulinemia (Clic here for more details about this article) |
7/9. Ocular copper deposition associated with benign monoclonal gammopathy and hypercupremia.The deposition of copper on Descemet's membrane and the anterior and posterior lens capsule with extreme hypercupremia and IgG hypergammaglobulinemia has been previously described with multiple myeloma and pulmonary carcinoma. A 66-year-old man presenting with blurred vision was found to have bilateral golden-brown metallic dust-like deposits on the central region of Descemet's membrane and the anterior and posterior lens capsule. Laboratory investigations revealed an elevated serum copper level 10 times the normal level associated with a monoclonal gammopathy and a normal ceruloplasmin level. copper binding to the serum proteins was investigated by three biochemical methods. The results demonstrated that the major copper binding fraction in the serum was IgG. N-terminal amino acid analysis of the IgG did not find the sequence of Asp-Ala-His, which has been shown to be a copper binding site in albumin. This is the first report of benign monoclonal gammopathy being associated with the ocular deposition of copper.- - - - - - - - - - ranking = 0.16666666666667keywords = hypergammaglobulinemia (Clic here for more details about this article) |
8/9. Case report of a monoclonal gammopathy in a patient with chronic hepatitis: effects of beta-IFN treatment.A sixty-one-year-old woman, with cirrhosis, presented with a monoclonal gammopathy of uncertain significance (MGSU). Often in a condition of cirrhosis is present a benign M component hypergammaglobulinemia. The electrophoresis and the immunophoresis showed a dense papraprotein in the gamma-region, an IgG with K light chain, an uncertain Bence-Jones proteinuria, a medullary plasmacytosis (9%), and a following growth of paraprotein were present. Lymphoblastic plasma cell were absent. Treatment with beta-IFN 6 MU for a period of six months and 3 MU for a further period of three months proved ineffective for hepatic disease, but produced a quantitative reduction in gamma-G globulin, the Bence-Jones proteinuria was absent, a reduction in M component and in medullary plasmacytosis. electrophoresis showed a polyclonal evolution of the gammopathy. Suspension of treatment was followed by de novo rise of monoclonal immunoglobulin. The authors report the use of beta-IFN in the therapy of multiple myeloma.- - - - - - - - - - ranking = 0.16666666666667keywords = hypergammaglobulinemia (Clic here for more details about this article) |
9/9. immunoblastic lymphadenopathy-like T cell lymphoma evolving into a massive plasma cell proliferation with biclonal paraproteinemia.We present a case of immunoblastic lymphadenopathy-like T cell lymphoma (IBL-T) who subsequently developed a massive proliferation of plasma cells. At diagnosis of IBL-T, the patient had polyclonal hypergammaglobulinemia and subsequently, while on chemotherapy, developed paraproteinemia with biclonal peaks and the IBL-T lesion was replaced with a massive proliferation of CD38-positive plasma cells. The evolution was not likely to be attributed to a new neoplastic proliferation of B cells. It appeared that two B cell clones possibly had a growth advantage among the polyclonal B cells due to a depletion of suppressor T cells or to a disturbance in the immune system.- - - - - - - - - - ranking = 0.16666666666667keywords = hypergammaglobulinemia (Clic here for more details about this article) |