ABSTRACT
INTRODUCTION: The kidney is one of the organs most frequently affected by disease processes which produce monoclonal immunoglobins, therefore renal morphological and immunopathological alterations should be clearly recognized. OBJECTIVE: To describe the pathological features of renal involvement in monoclonal gammopathies. MATERIAL AND METHODS: A descriptive, retrospective and cross-sectional study of renal biopsies studied in a single center during a period of 14 years was carried out. RESULTS: 102 cases were included, of which 53% were male patients and the median age was 62.5 years (range 34 - 79). 97% of the biopsies were from native kidneys. The most frequent histopathological diagnosis (31.4%) was myeloma kidney, with kappa being the light chain most frequently deposited (65.6% of cases). AL amyloidosis was the second most common (29.4%) where the lambda chain predominated in 86.6%, followed by light chain deposition disease (20.6%) with the predominance of the kappa chain in 66.6%. CONCLUSIONS: The most frequent renal involvement due to monoclonal gammopathies was myeloma kidney with deposition of kappa light chains, followed by AL lambda amyloidosis; these diseases were found more frequently in patients over 50 years of age.
Subject(s)
Kidney Diseases , Multiple Myeloma , Paraproteinemias , Adult , Aged , Cross-Sectional Studies , Humans , Kidney Diseases/pathology , Male , Middle Aged , Paraproteinemias/complications , Paraproteinemias/pathology , Retrospective StudiesABSTRACT
Introducción: El riñón es uno de los órganos más comprometidos en enfermedades con producción de inmunoglobulinas monoclonales, por lo que es de gran importancia caracterizar muy bien las alteraciones morfológicas e inmunopatológicas en dicho compromiso. Objetivo: Describir las características del compromiso renal en gammapatías monoclonales desde una perspectiva anatomopatológica. Material y métodos: Se realizó un estudio descriptivo, retrospectivo y transversal, recolectándose biopsias renales estudiadas en un único centro durante un período de 14 años. Resultados: Se incluyeron 102 casos provenientes de 102 pacientes, el 53% de sexo masculino, la mediana de la edad fue 62,5 años (rango 34-79). El diagnóstico histopatológico más frecuente fue riñón de mieloma (31,4% de los casos), siendo kappa la cadena ligera más frecuentemente depositada (65,6% de casos), seguido de amiloidosis AL (29,4%), donde predominó la cadena lambda en un 86,6% y la enfermedad por depósitos de cadenas ligeras (20,6%) con predominio de la cadena kappa: 66,6%. El 97% de las biopsias fueron de riñón nativo. Conclusiones: El compromiso renal por gammapatías monoclonales más frecuente fue el riñón de mieloma con depósito de cadenas ligeras kappa, seguida de la amiloidosis AL lambda; estas enfermedades se encontraron con mayor frecuencia en pacientes mayores de 50 años de edad.(AU)
Introduction: The kidney is one of the organs most frequently affected by disease processes which produce monoclonal immunoglobins, therefore renal morphological and immunopathological alterations should be clearly recognized. Objective: To describe the pathological features of renal involvement in monoclonal gammopathies. Material and methods: A descriptive, retrospective and cross-sectional study of renal biopsies studied in a single center during a period of 14 years was carried out. Results: 102 cases were included, of which 53% were male patients and the median age was 62.5 years (range 34 - 79). 97% of the biopsies were from native kidneys. The most frequent histopathological diagnosis (31.4%) was myeloma kidney, with kappa being the light chain most frequently deposited (65.6% of cases). AL amyloidosis was the second most common (29.4%) where the lambda chain predominated in 86.6%, followed by light chain deposition disease (20.6%) with the predominance of the kappa chain in 66.6%. Conclusions: The most frequent renal involvement due to monoclonal gammopathies was myeloma kidney with deposition of kappa light chains, followed by AL lambda amyloidosis; these diseases were found more frequently in patients over 50 years of age.(AU)
Subject(s)
Humans , Adult , Middle Aged , Aged , Paraproteinemias/complications , Kidney Diseases , Multiple Myeloma , Immunoglobulin Light-chain Amyloidosis , KidneyABSTRACT
El Mieloma Múltiple (MM) es la gammapatia monoclonal (GM) que más frecuentemente compromete la función renal, no obstante, un número creciente de enfermedades renales asociadas a GM están siendo reconocidas. Las GM son un conjunto de entidades caracterizadas por la producción anómala y secreción a la sangre de una inmunoglobulina (Ig) monoclonal (de un mismo clon de células plasmáticas) o un fragmento de la misma (cadenas pesadas, ligeras o ambas) que puede depositarse en los órganos de forma organizada como cristales, fibrillas o microtúbulos, o de forma no organizada (granular). Esta Ig llega a depositarse principalmente en el riñón, no sólo porque es un órgano muy vascularizado, sino también porque el túbulo renal tiene un papel predominante en el metabolismo de las Igs. El diagnóstico del compromiso renal ha sufrido cambios en las últimas décadas, siendo cada vez más certero debido al desarrollo e implementación rutinaria de distintas técnicas de laboratorio (tinciones con anticuerpos específicos contra cadenas ligeras kappa y lambda, estudio con microscopia electrónica (ME) y el desarrollo de técnicas cada vez más sensibles para detectar el componente monoclonal en sangre u orina). El compromiso renal en estas patologías frecuentemente se asocia a GM malignas, generalmente asociadas a depósitos de cadenas ligeras. Cada vez son más los casos de disfunción renal asociados a GM, y en muchos de estos es evidente el progreso a enfermedad renal terminal y con altas tasas de recurrencia después del transplante renal.
Multiple myeloma (MM) is the monoclonal gammopathy (MG) that most frequently compromises renal function; however, a growing number of renal diseases associated with monoclonal gammopathies are being recognized. Monoclonal gammopathies (MG) are a set of entities characterized by the abnormal production and secretion into the blood of a monoclonal immunoglobulin (Ig) from a single clone of plasma cells or a fragment thereof (heavy, light chains orboth) which can be deposited in the organs in an organized way such as crystals, fibrils or microtubules, orin an unorganized (granular) form. This Ig is mainly deposited in the kidney, not only because it is a very vascularized organ, but also because the renal tubule plays a predominant role in the metabolism of Igs. The diagnosis of renal involvement has undergone changes in the last decades, being more and more certain due to the development and routine implementation of different laboratory techniques (stains with specific antibodies against kappa and lambda light chains, study with electron microscopy (EM), development of increasingly sensitive techniques for detecting the monoclonal component in blood or urine). Renal involvement in these pathologies is often associated with malignant MG, generally associated with light chain deposits. Increasingly, there are cases of renal dysfunction associated with MG, and progression to terminal renal disease and high rates of recurrence after renal transplantation are evident in many of these.
Subject(s)
Humans , Kidney Diseases , Muscle Hypotonia/congenitalABSTRACT
La nefropatía asociada a las gammapatías monoclonales es debida principalmente al depósito de cadenas ligeras. Las enfermedades renales paraproteinémicas son lesiones asociadas con depósito de inmunoglobulinas intactas o fragmentos de inmunoglobulinas (cadenas pesadas y cadenas ligeras). La enfermedad por depósito de cadenas ligeras es una condición rara, caracterizada por el depósito de cadenas ligeras monoclonales en muchos órganos y a nivel renal predominantemente en glomérulos y membranas basales tubulares. La enfermedad está frecuentemente asociada con desórdenes linfoproliferativos, y la mayoría de casos son causados por depósito de cadenas ligeras kappa. Aunque se presenta sobre todo en cuadros malignos, en ocasiones no se detecta patología hematológica y se denomina idiopática o "primaria". Suele manifestarse como una insuficiencia renal severa con proteinuria nefrótica, no tiene tratamiento claramente establecido y el pronóstico es malo. Se describen las características clínicas e histológicas del segundo caso informado en Colombia de nefropatía por depósito de cadenas ligeras diagnosticado en el contexto de una enfermedad renal paraproteinémica sin datos de malignidad. (Acta Med Colomb 2014; 39: 196-201).
Nephropathy associated with monoclonal gammopathies is mainly due to light chain deposition. The paraproteinemic kidney diseases are lesions associated with deposition of intact immunoglobulins or fragments of immunoglobulins (heavy and light chains). The disease due to deposition of light chains is a rare condition characterized by deposition of monoclonal light chains in many organs and as for the kidney, predominantly in glomeruli and tubular basement membranes. The disease is frequently associated with lymphoproliferative disorders and the majority of cases are caused by deposition of kappa light chains. Although presented primarily in clinical pictures of malignancy, sometimes no hematological pathology is detected and is called idiopathic or "primary". It usually manifests as severe renal failure with nephrotic proteinuria, has not a clearly established treatment and the prognosis is poor. The clinical and histological features of the second case reported in Colombia of a light chain deposition nephropathy diagnosed in the context of a kidney paraproteinemic disease without malignancy data, is presented. (Acta Med Colomb 2014; 39: 196-201).
