RESUMO
Multiple myeloma (MM) is characterized by abnormal proliferation of clonal plasma cells or monoclonal plasmacytosis, resulting in accumulation of clonal immunoglobulins. Monoclonal gammopathy of unknown significance (MGUS) is considered a premorbid stage for developing MM. Studies have shown an increased risk of MGUS in first-degree relatives of patients with MM. Detection of immunoglobulin heavy chain gene (IGH) rearrangement provides a useful tool for assessing clonality. The aim of this study was to determine clonality in peripheral blood samples from 61 healthy first-degree relatives of MM probands by sorting circulating lymphocytes and detection of the IGH rearrangements in these cells. We detected 16 out of 61 (26.2%) relatives with monoclonal complete and incomplete IGH rearrangements; only three of them showed elevated monoclonal immunoglobulin in the serum protein electrophoresis. We conclude that this strategy is able to identify efficiently clonality in peripheral blood samples from first-degree relatives of patients with MM, who have a non-negligible risk of developing MGUS or other plasma cell dyscrasias.
Assuntos
Rearranjo Gênico , Cadeias Pesadas de Imunoglobulinas/sangue , Mieloma Múltiplo/imunologia , ADP-Ribosil Ciclase 1/metabolismo , Adolescente , Adulto , Idoso de 80 Anos ou mais , Proliferação de Células , Separação Celular , Eletroforese Capilar , Saúde da Família , Feminino , Citometria de Fluxo , Humanos , Cadeias Pesadas de Imunoglobulinas/genética , Masculino , Pessoa de Meia-Idade , Gamopatia Monoclonal de Significância Indeterminada/imunologia , Células Neoplásicas Circulantes , Plasmócitos/citologiaRESUMO
The diagnosis of T-cell large granular lymphocytic leukemia in association with other B-cell disorders is uncommon but not unknown. However, the concomitant presence of three hematological diseases is extraordinarily rare. We report an 88-year-old male patient with three simultaneous clonal disorders, that is, CD4+/CD8(weak) T-cell large granular lymphocytic leukemia, monoclonal gammopathy of unknown significance and monoclonal B-cell lymphocytosis. The patient has only minimal complaints and has no anemia, neutropenia or thrombocytopenia. Lymphadenopathy and hepatosplenomegaly were not present. The three disorders were characterized by flow cytometry analysis, and the clonality of the T-cell large granular lymphocytic leukemia was confirmed by polymerase chain reaction. Interestingly, the patient has different B-cell clones, given that plasma cells of monoclonal gammopathy of unknown significance exhibited a kappa light-chain restriction population and, on the other hand, B-lymphocytes of monoclonal B-cell lymphocytosis exhibited a lambda light-chain restriction population. This finding does not support the antigen-driven hypothesis for the development of multi-compartment diseases, but suggests that T-cell large granular lymphocytic expansion might represent a direct antitumor immunological response to both B-cell and plasma-cell aberrant populations, as part of the immune surveillance against malignant neoplasms.
Assuntos
Leucemia Linfocítica Granular Grande/complicações , Linfocitose/complicações , Gamopatia Monoclonal de Significância Indeterminada/complicações , Idoso de 80 Anos ou mais , Separação Celular , Citometria de Fluxo , Humanos , Leucemia Linfocítica Granular Grande/imunologia , Leucemia Linfocítica Granular Grande/patologia , Linfocitose/imunologia , Linfocitose/patologia , Masculino , Gamopatia Monoclonal de Significância Indeterminada/imunologia , Gamopatia Monoclonal de Significância Indeterminada/patologia , Reação em Cadeia da PolimeraseRESUMO
Os anticorpos contra glicolípides e glicoproteínas ocorrem em títulos elevados em doenças do neurônio motor e neuropatias periféricas. Inicialmente foram observados em neuropatias crônicas desmielinizantes com anti-corpos séricos de classe IgM que aparecem contra a glicoproteína da mielina (MAG). Os anticorpos contra gangliosídeos, fraçäo GMI, aparecem freqüentemente, também, em doenças do neurônio motor e neuropatias periféricas. Os anticorpos contra sulfatídeos podem ocorrer em polineuropatias idiopáticas, axonais, sensitivo-motoras, porém predominantemente sensitivas. Os anticorpos contra a proteína de desenvolvimento neuronal Hu, anti-Hu, se associam a glanglionopatias sensitivas paraneoplásicas; assim como as polineuropatias paraneoplásicas frequentemente revelam anticorpos contra o antígeno p-26
Assuntos
Humanos , Doença dos Neurônios Motores/imunologia , Glicoproteínas/imunologia , Glicolipídeos/imunologia , Doenças do Sistema Nervoso Periférico/imunologia , Anticorpos Monoclonais/imunologia , Gangliosídeo G(M1) , Gamopatia Monoclonal de Significância Indeterminada/imunologia , Síndrome POEMS/imunologiaRESUMO
Monoclonial gammopathies can either be benign or more commonly malignant. The commonest disease associated with it is multiple myeloma. Over the seven-year period 1984-1990, two hundred and thirty-four monoclonal gammopathies were seen at the University Hospital, Jamaica. Multiple myeloma was diagnosed in one hundred and fifty-six cases (84 males and 72 females). The diagnosis of most of the others were not known as the samples came from other institution. Of the patients with myeloma, the most common immunoglobulin type was IgG followed by IgA and then pure light chain disease. Only in about half of the cases where urine was analysed was Bence-Jones protien found. The majority of the cases had abnormal total serum protein, albumin and total globulin concentrations. Most of the cases also were in renal failure. Hypercalcaemia, hyperphoshataemia, elevated alkaline phosphate, gammaglutamyl transferase and aspartate aminotransferase occured in about one-third of them. These results were not much different from those reported in other countries
Assuntos
Humanos , Mieloma Múltiplo/metabolismo , Gamopatia Monoclonal de Significância Indeterminada/imunologia , Gamopatia Monoclonal de Significância Indeterminada/metabolismo , Mieloma Múltiplo/diagnóstico , Mieloma Múltiplo/imunologiaRESUMO
Monoclonial gammopathies can either be benign or more commonly malignant. The commonest disease associated with it is multiple myeloma. Over the seven-year period 1984-1990, two hundred and thirty-four monoclonal gammopathies were seen at the University Hospital, Jamaica. Multiple myeloma was diagnosed in one hundred and fifty-six cases (84 males and 72 females). The diagnosis of most of the others were not known as the samples came from other institution. Of the patients with myeloma, the most common immunoglobulin type was IgG followed by IgA and then pure light chain disease. Only in about half of the cases where urine was analysed was Bence-Jones protien found. The majority of the cases had abnormal total serum protein, albumin and total globulin concentrations. Most of the cases also were in renal failure. Hypercalcaemia, hyperphoshataemia, elevated alkaline phosphate, gammaglutamyl transferase and aspartate aminotransferase occured in about one-third of them. These results were not much different from those reported in other countries. (AU)
Assuntos
Humanos , Mieloma Múltiplo/metabolismo , Mieloma Múltiplo/diagnóstico , Mieloma Múltiplo/imunologia , Gamopatia Monoclonal de Significância Indeterminada/imunologia , Gamopatia Monoclonal de Significância Indeterminada/metabolismoAssuntos
Imunidade , Antígenos/isolamento & purificação , Anticorpos/isolamento & purificação , Antígenos/imunologia , Anticorpos/imunologia , Proteínas do Sistema Complemento/imunologia , Reações Antígeno-Anticorpo , Complexo Principal de Histocompatibilidade , Hipersensibilidade , Hipersensibilidade Tardia , Imunidade Celular , Inflamação/imunologia , Linfócitos T/imunologia , Linfócitos B/imunologia , Gamopatia Monoclonal de Significância Indeterminada/imunologia , Doenças Autoimunes , Micoses/imunologia , Viroses/imunologia , /métodos , Anticorpos Monoclonais , Neoplasias , Hibridomas , Ensaio de Imunoadsorção Enzimática/métodos , Técnicas Imunológicas , /métodos , Imunoeletroforese/métodos , Imunodifusão/métodos , Cromatografia por Troca Iônica/métodos , Soros Imunes/biossíntese , Radioimunoensaio/métodos , Eletroforese em Gel de Poliacrilamida/métodos , /métodos , Antígenos de Bactérias/isolamento & purificação , Antígenos de Bactérias/imunologiaAssuntos
Anticorpos/isolamento & purificação , Antígenos/isolamento & purificação , Imunidade , Anticorpos Monoclonais , Anticorpos/imunologia , Antígenos de Bactérias/imunologia , Antígenos de Bactérias/isolamento & purificação , Antígenos/imunologia , Complexo Principal de Histocompatibilidade , Cromatografia por Troca Iônica/métodos , Doenças Autoimunes , Eletroforese em Gel de Poliacrilamida/métodos , Ensaio de Imunoadsorção Enzimática/métodos , Gamopatia Monoclonal de Significância Indeterminada/imunologia , Hibridomas , Hipersensibilidade , Hipersensibilidade Tardia , Imunidade Celular , Imunodifusão/métodos , Imunoeletroforese/métodos , Inflamação/imunologia , Linfócitos B/imunologia , Linfócitos T/imunologia , Micoses/imunologia , Neoplasias , Proteínas do Sistema Complemento/imunologia , Radioimunoensaio/métodos , Reações Antígeno-Anticorpo , Soros Imunes/biossíntese , Técnicas Imunológicas , Viroses/imunologiaRESUMO
Se analizan 26 casos de gammapatías monoclonales, presentados en pacientes del Hospital Regional de talca, en los últimos seis años. Se muestra el tipo de patología y frecuencia con que se presentaron 21 casos de gammapatía monoclonal maligna (G.M.M.) y 5 casos de gammapatía monoclonal benigna (G.M.B.). La distribución según sexo y edad es analizada en cada grupo. Se muestran las características inmunológicas (clase de inmunoglobulina, migración electroforética y nivel sérico de componente monoclonal) y hematológicas (anemia, leucopenia y trombopenia) presentadas en las G.M.N. y G.M.B. estudiadas