A case report of multiple myeloma complicated with primary systemic light amyloidosis peripheral neuropa-thy / 中国神经精神疾病杂志

This paper reports a case of multiple myeloma complicated with Primary systemic light chain amyloidosis peripheral neuropathy.Patient was a male,60 years old with subacute onset of peripheral neuropathy.Patient had previous history of hypertension,coronary heart disease and stable angina pectoris.His urine routine examination 2 months ago showed urine protein(+++).The first clinical symptom with peripheral neuropathy characterized by progressive numbness and weakness of both lower limbs.The diagnosis was made after completing electromyography,lumbar puncture,renal puncture,bone puncture and other related examinations,and relevant treatment was given.As a rare disease of nervous system,the incidence rate is low.Early diagnosis is an important step to reduce mortality and improve prognosis.However,the disease is easily misdiagnosed as diabetic peripheral neuropathy and chronic inflammatory demyelinating polyradiculoneuropathy,thereby delaying diagnosis and treatment.

Diagnóstico de amiloidosis renal AA mediante espectrometría de masas en un paciente con proceso inflamatorio crónico grave abdominal

Introducción la amiloidosis es una enfermedad rara, producto del plegamiento y depósito normal de proteínas en tejidos y órganos. Esta enfermedad puede tener un compromiso renal que se manifiesta con síndrome nefrótico y deterioro de la función renal y su etiología puede estar asociada a amiloidosis con compromiso sistémico, siendo la amiloidosis AL y la amiloidosis AA las más frecuentes, esta última está asociada a inflamación crónica grave de origen infecciosa o autoinmune. Para el diagnóstico es fundamental el estudio sistémico multidisciplinario (hematológico, cardiaco, autoinmune, infeccioso y neoplásico), y cuando hay compromiso renal: la biopsia con estudio completo de microscopía de luz, tinciones especiales incluyendo rojo congo, inmunofluorescencia y microscopía electrónica. Cuando no se logra establecer la causa, la espectrometría de masas es una ayuda crucial para el diagnóstico específico. Objetivo se presenta el caso de un paciente con un proceso inflamatorio crónico grave abdominal que evolucionó a síndrome nefrótico por amiloidosis AA, donde la espectrometría de masas ayudó a aclarar el diagnóstico. Presentación del caso se presenta el caso de un paciente con un proceso inflamatorio crónico grave abdominal que evolucionó a síndrome nefrótico por amiloidosis AA, donde la espectrometría de masas ayudó a aclarar el diagnóstico Discusión y conclusiones se considera que la espectrometría de masas es un estudio diagnóstico muy importante para establecer el diagnóstico etiológico de la amiloidosis cuando otros métodos no han logrado establecerlo.

Introduction Amyloidosis is a rare disease, resulting from the accumulation and deposition of insoluble proteins in tissues or organs. This disease may involve the kidney, resulting in nephrotic syndrome and renal failure. The amyloidosis has been associated with systemic involvement, with AL amyloidosis and AA amyloidosis being the most common. The last is associated with various inflammatory disorders as chronic infections and autoimmune diseases. A multidisciplinary approach is required to the diagnosis (hematologic, cardiac, autoimmune, infectious, neoplastic) and in cases of renal involvement, a kidney biopsy with complete study of light microscopy, special stains including congo red, immunofluorescence, electron microscopy is essential for diagnosis. In cases where the cause cannot be stablished, mass spectrometry is practical tool to the identification of the correct type of amyloidosis. Purpose Here, we present a patient with a chronic and severe abdominal inflammatory process that progressed to a nephrotic syndrome due to AA amyloidosis, in which mass spectrometry helped to clarify the diagnosis. Case presentation Here, we present a patient with a chronic and severe abdominal inflammatory process that progressed to a nephrotic syndrome due to AA amyloidosis, in which mass spectrometry helped to clarify the diagnosis Discussion and conclusion Mass spectrometry is considered a useful diagnostic test to confirm the etiology of amyloidosis, especially if other methods are insufficient to establish it.

Primary Amyloidosis Presenting Nephrotic Syndrome and Hemorrhagic Gastritis / 대한신장학회잡지

A case of a 58 year old woman who was found to have primary amyloidosis is reported. She was presented with nephrotic syndrome and hemorrhgic gastritis. She was confirmed by showing apple green birefringence under the polarized microscope with Congo-red stain through renal and stomach biopsy. Immunoelectrophoresis of urine exhibited Bence-Jones protein(gamma type) and protein electrophoresis of urine exhibited monoclonal gammopathy. She was treated with cyclophosphamide and prednisolone, and proteinuria and gastrointestinal symptoms were improved. We discussed this case with a brief review of literature.