Cases reported "Hypergammaglobulinemia"

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1/74. Immunological reconstitution by allogeneic bone marrow transplantation in a child with the X-linked hyper-IgM syndrome.

    A successful transplantation of sibling marrow in a patient with the X-linked hyper-IgM syndrome is reported. Engraftment of HLA-identical marrow cells was obtained, although complicated by grade I acute graft-versus-host disease. Expression of the cd40 ligand (CD40L, CD154) by activated T-cells from the recipient remained at low levels until 10 months after the transplantation, but then normalized. The patient is now fully competent in immune function without any episodes of severe infection 24 months later. CONCLUSION: Allogeneic bone marrow transplantation is a reasonable therapeutic option for X-linked hyper-IgM syndrome if HLA-matched family donors are available. Whether dysregulation of CD40L expression causes post-transplant immunological abnormalities remains to be clarified.
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2/74. Posterior corneal crystalline deposits in benign monoclonal gammopathy: a clinicopathologic case report.

    A 74-year-old woman had bilateral, deep stromal, patchy crystalline corneal deposits with the greatest density in the midperiphery. visual acuity was 6/120 in the right eye and finger counting at 1 m in the left eye. Histological examination of the corneal button showed large, irregular amorphous masses in the posterior stroma. The deposits stained red with Masson's trichrome and were positive for protein with the Danielli stain. Stains for amyloid, copper, and lipid were negative. The immunoperoxidase stain was positive for polyvalent IgG and kappa light chains. Transmission electron microscopy disclosed electron-dense deposits with linear and honeycomb profiles. Laboratory investigations disclosed elevated serum and urinary IgG kappa light chain (bence jones protein) levels. Urinary amino acids were normal. The serum copper level was elevated. Antinuclear antibody was positive at a titer of 1:80. A bone marrow aspirate was normal, as were roentgenograms of the skull.
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3/74. Successful bone marrow transplantation in a child with X-linked hyper-IgM syndrome.

    We report a case of an 11-year-old boy who underwent successful bone marrow transplantation for X-linked hyper-IgM syndrome (XHIM). The donor was an HLA-matched brother. The patient was conditioned with busulfan, cyclophosphamide and anti-thymocyte globulin. He received 4.7 x 10(8) marrow cells per kg from the donor. Prophylaxis against graft-versus-host disease consisted of cyclosporine and short-term methotrexate. The clinical course after the bone marrow transplantation was uneventful, and 12 months after transplantation the patient was doing well with no need for therapy. We examined expression of the cd40 ligand (CD40L) on the patient's activated T lymphocytes and in vitro production of immunoglobulins by his lymphocytes. Although expression of CD40L was totally absent before the bone marrow transplant, subnormal expression appeared after the transplantation. in vitro production of IgG and IgA also was improved by the transplant. Based on our experience bone marrow transplantation appears to be a reasonable therapeutic option for patients with XHIM if HLA-matched family donors are available.
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4/74. Systemic AL amyloidosis in gaucher disease. A case report and review of the literature.

    Chronic gaucher disease [GD] in association with systemic AL amyloidosis is extremely rare. We describe a 46-year-old Greek male with chronic GD confirmed by low glucocerebroside activity in fibroblasts and N370S/L444P mutations at the cerebrosidase gene, who also had systemic AL amyloidosis diagnosed by bone marrow diffuse plasmacytosis, serum monoclonal IgA-lambda, severe total proteinuria with monoclonal IgA-lambda, Bence-Jones-lambda and amyloid deposits in bone marrow, liver, spleen and kidney biopsy specimens. Treatment with melphalan and prednizolon has dramatically decreased both levels of serum M-IgA and proteinuria and also improved the clinical symptoms of amyloidosis. He died from restrictive cardiac disease 30 months after the diagnosis of amyloidosis. Previously reported cases of GD in association with AL amyloidosis are reviewed.
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5/74. A surgical case of solitary plasmacytoma of rib origin with biclonal gammopathy.

    Localized solitary plasmacytoma of the bone (SPB) is a rare disease and is characterized by only one or two isolated bone lesions with no evidence of disease dissemination. A previously healthy 44-year-old male was admitted for evaluation of an abnormal radiographic shadow in the left middle lung field with symptoms of left back pain. Radiological evaluation revealed a peripheral opacity in the left chest wall, which was highly suspected to be a chest wall tumor. CT-guided transcutaneous needle biopsy of the tumor was performed and the specimens showed a monomorphous population of mature plasma cells. The bone marrow biopsy findings revealed no evidence of myeloma and bone scanning revealed only abnormal accumulation in the left seventh rib. He had mild M-proteins in a urine sample and Bence-Jones protein was detected. immunoelectrophoresis revealed mild biclonal gammopathy of Bence-Jones protein of both the kappa and lambda light-chain types. Under a diagnosis of solitary bone plasmacytoma, preoperative radiation therapy with doses of 40 Gy for the tumor was performed. He underwent complete en bloc resection of the chest wall, including one-third of the left sixth and seventh ribs, the intercostal muscle and the parietal pleura. The protein abnormalities in the urine sample disappeared following surgical resection. Adjuvant chemotherapy using melphalan and prednisolone was performed. He is doing well without evidence of tumor recurrence 2 years following his initial diagnosis.
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6/74. Spontaneous B-cell IgE production in a patient with remarkable eosinophilia and hyper IgE.

    BACKGROUND: The pathophysiology of eosinophilia and hyper-IgE is not fully elucidated yet. OBJECTIVE: To clarify the pathophysiology of a patient with remarkable eosinophilia and hyper IgE, we examined cytokine levels in serum, surface antigens of peripheral blood eosinophils and IgE production in vitro. RESULTS: Concentrations of tumor necrosis factor-alpha (TNF-alpha), interleukin-3 (IL-3), interleukin-4 (IL-4), interleukin-5 (IL-5), and granulocyte/macrophage-colony stimulating factor (GM-CSF) in the serum were 21 pg/mL, <15 pg/mL, <15 pg/mL, 8 pg/mL, and <5 pg/mL pg/mL, respectively. Newly expressed surface antigens CD4, CD25, CD69, and HLA-DR, but not CD54, were observed on peripheral blood eosinophils. Extremely high levels of IgE secretion was found in the patient's mononuclear cells without stimuli; this was not enhanced by IL-4 or IL-4 plus anti-CD40 monoclonal antibody stimulation. Furthermore, highly purified B cells spontaneously produced large amounts of IgE and the production was not enhanced in addition of his T cells. CONCLUSION: The eosinophils were activated, and the B cells spontaneously produced IgE independently of T cells or cytokines, suggesting that intrinsic abnormality of B cells leading to dysregulated production of IgE in this disease.
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7/74. Biclonal gammopathy and platelet antibodies in a patient with chronic hepatitis c virus infection and mixed cryoglobulinemia.

    The presence of biclonal gammopathy [immunoglobulin (Ig)Mk IgAlambda] and platelet antibodies in a patient with chronic hepatitis c virus (HCV) infection is reported. A type-II cryoglobulinemia (IgMk and polyclonal IgG) has also been detected. The IgAlambda monoclonal component still persists after removal of the cryoprecipitate. Two populations of atypical lymphoid cells with plasmocytoid features in bone marrow have been detected. They express IgM with k restriction and IgA with lambda restriction, respectively. These cells are CD19 , CD5 , bc12 , and Ki67-. liver biopsy shows lymphoid infiltrates with features of B follicles. thrombocytopenia with platelet antibodies but without splenomegaly is also present. This case is interesting because two hematologic extrahepatic manifestations simultaneously occur in a patient with HCV infection. Moreover the association between HCV and biclonal gammopathies has never been reported.
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8/74. 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.
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9/74. association of a poems syndrome and light chain deposit disease: first case report.

    Monoclonal immunoglobulin (Ig) deposition diseases are characterized by deposition in tissues of excessive amounts of the Ig, compromising organ functions. light chain deposition disease (LCDD) and AL amyloidosis are the commonest [Buxbaum 1992]. LCDD is usually characterized by rapidly progressive renal failure with glomerular and tubular deposits of Ig fragments mostly composed by kappa light chain. Monoclonal Ig production can also be observed associated with various symptoms, that, taken together, have been described as the Crow-Fukase syndrome or poems syndrome. It associates polyneuropathy, organomegaly, endocrinopathy, monoclonal Ig, and skin changes. In poems syndrome, renal abnormalities are rare and are reported as a moderate renal insufficiency with mild proteinuria or acute functional renal insufficiency leading in some cases to end-stage renal failure [Fukatsu et al. 1991]. Although a monoclonal Ig is produced, no Ig deposit disease had been described in poems syndrome except a case of AL amyloidosis [Toyokuni et al. 1992]. Here, to our knowledge, we report the first case of an LCDD associated with a poems syndrome. Although an autologous bone marrow graft was realized, the monoclonal component reappeared and was responsible for end-stage renal disease, cachexia and death.
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10/74. 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.
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