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      Ocular copper deposition associated with monoclonal gammopathy of undetermined significance: case report Translated title: Depósito ocular de cobre associado a gamopatia monoclonal de significância indeterminada: relato de caso

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          Abstract

          To report a case of ocular copper deposition in both eyes at the level of Descemet's membrane associated with a monoclonal gammopathy of undetermined significance (MGUS). A 49-year-old white woman had golden-brown metallic dust-like deposits on Descemet's membrane of both eyes. A systemic examination revealed an elevated serum copper, normal serum ceruloplasmin and a normal level of total protein. Serum protein electrophoresis demonstrated a single peak (M-spike) in the gamma region (M-protein in serum = 11 g/l). Flow cytometric analysis of the marrow aspirate identified a monoclonal plasma cell population that represents approximately 2% of the total marrow cells consistent with the diagnosis of monoclonal gammopathy of undetermined significance. Copper deposits at the level of Descemet's membrane may be a finding in a patient with monoclonal gammopathy of undetermined significance.

          Translated abstract

          Relatar um caso de depósito corneano de cobre em ambos os olhos a nível da membrana de Descemet associado a gamopatia monoclonal de significância indeterminada (GMSI). Paciente feminina, 49 anos, leucodérmica, apresentando depósito corneano de aspecto marrom-ouro a nível da membrana de Descemet em ambos os olhos. Exame sistêmico revelou cobre sérico elevado, ceruloplasmina sérica normal, e proteína total normal. Eletroforese de proteínas séricas demonstrou um pico único na região gama (proteína M = 11 g/l). Análise citométrica de aspirado medular evidenciou uma população de células plasmáticas monoclonais de aproximadamente 2% do total de células medulares consistente com o diagnóstico de gamopatia monoclonal de significância indeterminada. Depósitos de cobre a nível da membrana de Descemet podem ser encontrados em pacientes com gamopatia monoclonal de significância indeterminada.

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          Most cited references18

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          Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group.

          (2003)
          The monoclonal gammopathies are a group of disorders associated with monoclonal proliferation of plasma cells. The characterization of specific entities is an area of difficulty in clinical practice. The International Myeloma Working Group has reviewed the criteria for diagnosis and classification with the aim of producing simple, easily used definitions based on routinely available investigations. In monoclonal gammopathy of undetermined significance (MGUS) or monoclonal gammopathy, unattributed/unassociated (MG[u]), the monoclonal protein is /= 30 g/l and/or bone marrow clonal cells >/= 10% but no related organ or tissue impairment (ROTI)(end-organ damage), which is typically manifested by increased calcium, renal insufficiency, anaemia, or bone lesions (CRAB) attributed to the plasma cell proliferative process. Symptomatic myeloma requires evidence of ROTI. Non-secretory myeloma is characterized by the absence of an M-protein in the serum and urine, bone marrow plasmacytosis and ROTI. Solitary plasmacytoma of bone, extramedullary plasmacytoma and multiple solitary plasmacytomas (+/- recurrent) are also defined as distinct entities. The use of these criteria will facilitate comparison of therapeutic trial data. Evaluation of currently available prognostic factors may allow better definition of prognosis in multiple myeloma.
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            Copper homeostasis in the mammalian system.

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              Long-term follow-up of 241 patients with monoclonal gammopathy of undetermined significance: the original Mayo Clinic series 25 years later.

              To determine the long-term outcome of patients with monoclonal gammopathy of undetermined significance (MGUS). We reviewed the medical records of 241 patients with MGUS who were examined at the Mayo Clinic in Rochester, Minn, between January 1, 1956, and December 31, 1970. Follow-up was 3579 person-years (median, 13.7 years; range, 0-39 years). Only 14 patients (6%) were alive and had no substantial increase of M protein at last follow-up; 138 patients (57%) died without evidence of multiple myeloma or a related disorder; a malignant lymphoplasma cell proliferative disorder developed in 64 patients (27%). The interval from diagnosis of MGUS to diagnosis of multiple myeloma or related disorder ranged from 1 to 32 years (median, 10.4 years). The median survival rate of study patients with MGUS was only slightly shorter than that of a comparable US population. Risk of progression of MGUS to lymphoplasma cell malignancy is indefinite and persists even after more than 30 years of follow-up, with no reliable predictors of malignant evolution.
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                Author and article information

                Journal
                abo
                Arquivos Brasileiros de Oftalmologia
                Arq. Bras. Oftalmol.
                Conselho Brasileiro de Oftalmologia (São Paulo, SP, Brazil )
                0004-2749
                1678-2925
                August 2005
                : 68
                : 4
                : 539-541
                Affiliations
                [04] orgnameWills Eye Hospital orgdiv1Refractive Surgery Department
                [02] Brasília DF orgnameHospital Oftalmológico de Brasília orgdiv1Serviço de Córnea e Catarata
                [05] Philadelphia PA orgnameJefferson Medical College USA
                [01] Belo Horizonte MG orgnameUniversidade Federal de Minas Gerais orgdiv1Faculdade de Medicina
                [03] Philadelphia Pennsylvania orgnameWills Eye Hospital orgdiv1Serviço de Córnea USA
                Article
                S0004-27492005000400021 S0004-2749(05)06800421
                3256dab6-3f3d-4bee-b5cc-dd5a091e6560

                This work is licensed under a Creative Commons Attribution 4.0 International License.

                History
                : 01 April 2005
                : 04 November 2004
                : 15 April 2005
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 19, Pages: 3
                Product

                SciELO Brazil

                Categories
                Case Reports

                Cobre,Eletroforese de proteínas sangüíneas,Paraproteinemias,Gama-globulins,Descemet's membrane,Blood protein electrophoresis,Copper,Gamaglobulina,Membrana de Descemet

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