RESUMO
Light chain-associated kidney compromise is frequent in patients with monoclonal gammopathies; it affects the glomeruli or the tubules, and its most common cause is multiple myeloma. It may develop after a kidney transplant due to recurrence of a preexisting multiple myeloma or it can be a de novo disease manifesting as graft dysfunction and proteinuria. A kidney biopsy is always necessary to confirm the diagnosis.We describe three cases of kidney graft dysfunction due to multiple myeloma in patients without presence of the disease before the transplant.
Assuntos
Transplante de Rim/efeitos adversos , Mieloma Múltiplo/etiologia , Disfunção Primária do Enxerto/etiologia , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biópsia , Transplante de Medula Óssea , Terapia Combinada , Evolução Fatal , Humanos , Cadeias Leves de Imunoglobulina/análise , Imunossupressores/efeitos adversos , Falência Renal Crônica/cirurgia , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/diagnóstico , Mieloma Múltiplo/terapia , Proteínas do Mieloma/análise , Proteinúria/etiologiaRESUMO
RESUMEN La enfermedad renal asociada a cadenas ligeras es frecuente en el contexto de las gammapatías monoclonales, afecta los glomérulos o los túbulos renales, y su causa más común es el mieloma múltiple. Puede desarrollarse después de un trasplante renal por recurrencia de un mieloma múltiple ya existente, o puede ser de diagnóstico nuevo y presentarse con deterioro de la función renal y proteinuria. Siempre se requiere una biopsia renal para confirmar el diagnóstico.
ABSTRACT Light chain-associated kidney compromise is frequent in patients with monoclonal gammopathies; it affects the glomeruli or the tubules, and its most common cause is multiple myeloma. It may develop after a kidney transplant due to recurrence of a preexisting multiple myeloma or it can be a de novo disease manifesting as graft dysfunction and proteinuria. A kidney biopsy is always necessary to confirm the diagnosis.
Assuntos
Idoso , Humanos , Masculino , Pessoa de Meia-Idade , Transplante de Rim/efeitos adversos , Disfunção Primária do Enxerto/etiologia , Mieloma Múltiplo/etiologia , Proteinúria/etiologia , Biópsia , Proteínas do Mieloma/análise , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Transplante de Medula Óssea , Cadeias Leves de Imunoglobulina/análise , Evolução Fatal , Terapia Combinada , Imunossupressores/efeitos adversos , Falência Renal Crônica/cirurgia , Mieloma Múltiplo/diagnóstico , Mieloma Múltiplo/terapiaAssuntos
Colestase Extra-Hepática/etiologia , Icterícia Obstrutiva/etiologia , Mieloma Múltiplo/complicações , Neoplasias Pancreáticas/complicações , Idoso , Anorexia/etiologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Colangiopancreatografia Retrógrada Endoscópica , Doenças do Ducto Colédoco/etiologia , Ciclofosfamida/administração & dosagem , Dexametasona/administração & dosagem , Diabetes Mellitus Tipo 1/etiologia , Diagnóstico por Imagem , Progressão da Doença , Humanos , Cadeias Leves de Imunoglobulina/análise , Lenalidomida , Masculino , Mieloma Múltiplo/patologia , Proteínas do Mieloma/análise , Recidiva , Indução de Remissão , Terapia de Salvação , Stents , Talidomida/administração & dosagem , Talidomida/análogos & derivadosRESUMO
Introducción: el mieloma múltiple (MM) es una enfermedad caracterizada por una proliferación monoclonal de inmunoglobulinas que representa aproximadamente el 15 por ciento de las hemopatías malignas. Métodos: se realizó un estudio de la distribución de las clases, sub clases y tipos de cadenas ligeras de inmunoglobulinas en 285 enfermos con el diagnóstico de MM. Se emplearon tres métodos: electroforesis de proteínas en suero para la detección de la inmunoglobulina monoclonal o paraproteína, electroforesis de inmunofijación y doble inmunodifusión para identificar las clases, sub clases y tipo de cadenas ligeras. Resultados: se encontraron 206 enfermos (72.28 por ciento) con MM IgG; 73 (25.62 por ciento) con MM IgA y 6 (2.1 por ciento) con MM IgM. La distribución de sub clases de IgG fue: 130 casos (63.11 por ciento) IgG1, 43 (20.87 por ciento) IgG2, 21 (10.19 porciento) IgG3 y 12 (5.83 por ciento) IgG4; y la de sub clases de IgA fue de 59 enfermos (80.82 por ciento) IgA1 y 14 (19.18 por ciento) IgA2. Del total de enfermos 187 (65.61 por ciento) mostraron cadenas ligeras tipo kappa y 98 (34.38 por ciento) tipo lambda. Conclusiones: los datos obtenidos en nuestro estudio permitieron identificar la frecuencia de distribución de las clases, subclases y cadenas ligeras en una muestra de enfermos con MM
Introduction: multiple mieloma (MM) is a disease characterized by a monoclonal proliferation of immunoglobulins representing approximately 15 percent of malignant hemopathies. Methods: the distribution of classes, subclasses and light chains of monoclonal immunoglobulins was studied in 285 patients with MM. Three methods were used: serum protein electrophoresis for the detection of monoclonal immunoglobulins or paraproteins, immunofixation electrophoresis and double immunodiffusion to identify classes, subclasses and light chain types. Results: 206 patients (72.28 percent) with IgG MM, 73 (25,62 percent) with IgA MM, and 6 (2,1 percent) with IgM MM were found. The distribution of IgG subclasses was: 130 cases (63,11 percent) IgG1; 43 (20,87 percent) IgG2; 21 (10,19 percent) IgG3: and 12 (5,83 percent) IgG4. Distribution of IgA subclasses was: 59 patients (80,82 percent) IgA1 and 14 (19,18 percent) IgA2; 187 patients (65,62 percent) showed kappa light chains and 98 (34,38 percent) were lambda. Conclusions: the data obtained in our study allowed us to identify the frequency of distribution of classes, subclasses and light chains in a sample of patients with MM
Assuntos
Hemoglobina A/análise , Mieloma Múltiplo/complicações , Paraproteínas/análise , Proteínas do Mieloma/análise , Cadeias kappa de Imunoglobulina/análise , Eletroforese das Proteínas Sanguíneas/métodos , Eletroforese/métodos , Cadeias Leves de ImunoglobulinaRESUMO
MATERIAL AND METHODS: In a 30-year period in a single institution, 23 cases of systemic immunoglobulin light chain amyloidosis (AL) were identified, within a group of 1,388 individuals with some form of a hematological malignancy. RESULTS: AL is 14 times less frequent in Mexico than in Caucasians and it represents 15% of all monoclonal gammopathies. Median age was 57 years (range 39-98); there were 11 males and 12 females. The histologic diagnosis was done in the periumbilical fat in 39%, the bone marrow in 30%, the kidney in 13%, the gastrointestinal tract in 13% and in a lymph node in one case. The nephrotic syndrome was present in 61% of cases, heart failure in 35%, sensorimotor peripheral neuropathy in 26% and weight loss in 6%. Anemia was present in 14% of cases at diagnosis; median hemoglobin was 11 g/dL. An abnormal monoclonal spike in the peripheral blood was present in 70% of cases; it had a median of 1.2 g/dL (range 0.2-3.6); there were 7 cases of light-chain only disease and five in whom an abnormal paraproteinemia was not found. Six cases were associated with overt multiple myeloma. Seventeen individuals (74%) were followed for more than 3 months (range 90 to 5190 days, median 210); their overall survival (OS) was 71% at 173 months, whereas the median OS has not been reached, being above 173 months. Eight patients were treated with melphalan/predisone and five were given high dose chemotherapy and an autologous stem cell transplantation; the others were given other treatments. CONCLUSIONS: AL is less frequent in Mexican mestizos and probably underrecognized; the clinical features of the disease are not significantly different from those informed from other populations.
Assuntos
Amiloidose/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Proteínas Amiloidogênicas/análise , Amiloidose/etnologia , Amiloidose/etiologia , Amiloidose/metabolismo , Amiloidose/patologia , Amiloidose/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ácidos Borônicos/administração & dosagem , Bortezomib , Dexametasona/administração & dosagem , Etnicidade/estatística & dados numéricos , Feminino , Transplante de Células-Tronco Hematopoéticas/estatística & dados numéricos , Humanos , Cadeias Leves de Imunoglobulina/análise , Masculino , Melfalan/administração & dosagem , México/epidemiologia , Pessoa de Meia-Idade , Gamopatia Monoclonal de Significância Indeterminada/complicações , Gamopatia Monoclonal de Significância Indeterminada/epidemiologia , Mieloma Múltiplo/complicações , Mieloma Múltiplo/mortalidade , Mieloma Múltiplo/terapia , Proteínas do Mieloma/análise , Especificidade de Órgãos , Prednisona/administração & dosagem , Estudos Prospectivos , Pirazinas/administração & dosagem , Talidomida/administração & dosagem , Resultado do Tratamento , Macroglobulinemia de Waldenstrom/complicações , Macroglobulinemia de Waldenstrom/epidemiologiaRESUMO
A 71-year-old female with primary biliary cirrhosis, multiple myeloma and hypothyroidism is reported. The liver biopsy showed stage II-III histologic damage according to Scheuer's classification and the multiple myeloma was IgG-lambda type, stage II-A at the time of diagnosis. Another three cases of primary biliary cirrhosis associated with multiple myeloma were found in the literature. As a group, the four cases had mild or moderate liver damage, extensive bone lesions and three of four cases had multiple myeloma IgG-lambda type. Although this association could be incidental, there is evidence that implies a pathogenic relationship. This information is summarized in this report.
Assuntos
Hipotireoidismo/complicações , Cirrose Hepática Biliar/complicações , Mieloma Múltiplo/complicações , Idoso , Transformação Celular Neoplásica , Feminino , Humanos , Hipergamaglobulinemia/etiologia , Imunoglobulina M/sangue , Cadeias lambda de Imunoglobulina/análise , Cirrose Hepática Biliar/imunologia , Ativação Linfocitária , Subpopulações de Linfócitos/imunologia , Subpopulações de Linfócitos/patologia , Proteínas do Mieloma/análise , Vitiligo/complicaçõesRESUMO
Beta 2 microglobulin is a low molecular weight protein found on the surface of all nucleated cells: it is the light chain of the HL-A histocompatibility complex. The increased levels of serum beta 2 microglobulin in patients with multiple myeloma have been associated with a poor prognosis. Pretreatment levels of serum beta 2 microglobulin were estimated in 70 previously untreated patients with multiple myeloma. In a multivariate analysis, serum beta 2 microglobulin levels and stage were the most significant prognostic factors for survival independent of other risk factors associated with a worse prognosis. There was a clear difference in survival duration observed between the patients with a high pretreatment level of beta 2 microglobulin and stage III (none alive at 5 years) compared with patients with normal levels and stage I (80% alive at five years) (p less than .001). We conclude that pretreatment-beta 2 microglobulin level is one of the most useful prognostic factors in patients with multiple myeloma.