ABSTRACT
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.
Subject(s)
Aged , Humans , Male , Middle Aged , Kidney Transplantation/adverse effects , Primary Graft Dysfunction/etiology , Multiple Myeloma/etiology , Proteinuria/etiology , Biopsy , Myeloma Proteins/analysis , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Bone Marrow Transplantation , Immunoglobulin Light Chains/analysis , Fatal Outcome , Combined Modality Therapy , Immunosuppressive Agents/adverse effects , Kidney Failure, Chronic/surgery , Multiple Myeloma/diagnosis , Multiple Myeloma/therapyABSTRACT
BACKGROUND/AIMS: The gastrointestinal (GI) tract often becomes involved in patients with systemic amyloidosis. As few GI amyloidosis data have been reported, we describe the clinical features and outcomes of patients with pathologically proven GI amyloidosis. METHODS: We identified 155 patients diagnosed with systemic amyloidosis between April 1995 and April 2013. Twenty-four patients (15.5%) were diagnosed with GI amyloidosis using associated symptoms, and the diagnoses were confirmed by direct biopsy. RESULTS: Among the 24 patients, 20 (83.3%) had amyloidosis light chain (AL), three (12.5%) had amyloid A, and one (4.2%) had transthyretin-related type amyloidosis. Their median age was 57 years (range, 37 to 72), and 10 patients were female (41.7%). The most common symptoms of GI amyloidosis were diarrhea (11 patients, 45.8%), followed by anorexia (nine patients, 37.5%), weight loss, and nausea and/or vomiting (seven patients, 29.2%). The histologically confirmed GI tract site in AL amyloidosis was the stomach in 11 patients (55.0%), the colon in nine (45.0%), the rectum in seven (35.0%), and the small bowel in one (5.0%). Patients with GI involvement had a greater frequency of organ involvement (p = 0.014). Median overall survival (OS) in patients with GI involvement was shorter (7.95 months; range, 0.3 to 40.54) than in those without GI involvement (15.84 months; range, 0.0 to 114.53; p = 0.069) in a univariate analysis. A multivariate analysis of prognostic factors for AL amyloidosis revealed that GI involvement was not a significant predictor of OS (p = 0.447). CONCLUSIONS: The prognosis of patients with AL amyloidosis and GI involvement was poorer than those without GI involvement, and they presented with more organ involvement and more advanced disease than those without organ involvement.
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Amyloid Neuropathies, Familial/diagnosis , Biomarkers/analysis , Biopsy , Gastrointestinal Diseases/diagnosis , Gastrointestinal Tract/immunology , Immunoglobulin Heavy Chains/analysis , Immunoglobulin Light Chains/analysis , Kaplan-Meier Estimate , Multivariate Analysis , Predictive Value of Tests , Prognosis , Proportional Hazards Models , Republic of Korea , Retrospective Studies , Risk Factors , Serum Amyloid A Protein/analysis , Time FactorsABSTRACT
El ensayo de cadenas livianas libres en suero cuantifica los niveles de κ y λ libres. Existen tres indicaciones principales para la medición de cadenas livianas libres en el manejo de pacientes con mieloma múltiple y enfermedades relacionadas. Primero, en el contexto del diagnóstico, donde en combinación con la electroforesis de proteínas en suero y la inmunofijación en suero proporcionan el esquema de tamizaje de mayor sensibilidad, eliminando la necesidad de analizar la orina de 24 horas. Segundo, los valores basales de cadenas livianas libres en suero han mostrado tener valor pronóstico para la mayoría de las gammapatías monoclonales. Tercero, el ensayo de cadenas livianas libres en suero permite un monitoreo cuantitativo y sensible en pacientes con presentación oligosecretora de la enfermedad, incluyendo la AL amiloidosis y cerca del 70% de los pacientes históricamente denominados no secretores. Esta revisión tiene por objeto actualizar la información sobre las aplicaciones de esta importante prueba...
Subject(s)
Humans , Amyloidosis , Immunoglobulin Light Chains/analysis , Multiple Myeloma , ParaproteinemiasABSTRACT
No abstract available.
Subject(s)
Humans , Male , Asymptomatic Diseases , Biomarkers/analysis , Biopsy , Crystallization , Fluorescent Antibody Technique , Immunoglobulin Light Chains/analysis , Kidney Diseases/diagnosis , Kidney Tubules, Proximal/immunology , Microscopy, Electron , Proteinuria/diagnosisABSTRACT
Idiopathic Light Chain disease (ILCD) is a systemic disease characterized by a deposit in different organs of light chain monoclonal immunoglobulins, produced by an abnormal clone ofB cells. It is usually found in the course ofa plasma cell dyscrasia and in other lymphoproliferative alterations; however it may occur in absence of any hematologic disease and is denominated as idiopathic. We report a 51-year-old mole admitted to the hospital due to anasarca. Laboratory evaluation showed a serum creatinine of 1.4 mg/dl, a serum albumin of1.6 g/dl, a serum cholesterol of 687 mg/dl and a proteinuria of 5.3 g/day Light chains with a predominance of a monoclonal component were identified in urinary proteins by electrophoresis and kappa chains were identified by immunofixation. A renal biopsy showed a diffuse nodular glomerulopathy with a 35% tubular atrophy and interstitial sclerosis. Electrón microscopy confirmed light chain deposition. The bone marrow biopsy showed a myeloid hyperplasia. Thepatient was initially treated with methylprednisolone and plasmapheresis with a reduction in serum creatinine and disappearance of urinary kappa component. Albuminuriapersisted and a malnutrition-inflammatory complex syndrome was diagnosed. Hemodialysis with ultrafiltration was started along with cyclophosphamide. Thepatient receivedhemodialysisforsixmonths and continued with methylprednisolone.
Subject(s)
Humans , Male , Middle Aged , Diabetic Nephropathies/etiology , Immunoglobulin Light Chains/analysis , Paraproteinemias/complications , Diabetic Nephropathies/pathology , Paraproteinemias/pathologyABSTRACT
Context: Light chain immunofluoresence (IF) in renal biopsy is routinely used in the diagnosis of light chain deposition disease (LCDD), amyloidosis and cast nephropathy. Light chain predominance has also been reported in certain glomerulopathies like IgA nephropathy. However, pathogenesis of this pattern of deposition in various glomerulopathies is uncertain. Aim: To discuss the pathogenesis and utility of light chain IF in nephropathies. Setting and Design: Retrospective study. Materials and Methods: The pattern of light chain IF and light microscopic diagnosis in 306 cases of various nephropathies was reviewed. Direct IF was done in all these cases with commercial fluorescence (Fluoresciene Isothiocynate ) conjugated polyclonal rabbit anti-human antisera against IgM, IgG, IgA, C3, C1q, kappa and lambda light chains. Results: Light chain deposits were seen in 240 (78.43%) cases. In IgA nephropathy, lupus nephritis and post-infectious glomerulonephritis (PIGN), lambda positivity was more as compared to kappa. Light chain deposits in LCDD and membranous nephropathy were more kappa type. The IF pattern in amyloidosis was not consistent. Conclusion: The pathogenesis of light chain predominance in glomerulopathies is not clear and it depends on isoelectric point and size of the immune complex. Light chain IF should be performed routinely in all the renal biopsies.
Subject(s)
Adolescent , Adult , Aged , Child , Child, Preschool , Complement C1q/analysis , Complement C3/analysis , Fluorescent Antibody Technique , Humans , Immunoglobulin Light Chains/analysis , Infant , Kidney Diseases/pathology , Male , Middle Aged , Retrospective Studies , Young AdultABSTRACT
We report a case of light chain deposition disease in a 59-yr-old female showing deposition of monoclonal light chain in the kidney and bone marrow accompanied with a schistocytosis, the morphologic finding of microangiopathic hemolytic anemia. The immunofluorescence examination of the kidney revealed strongly stained kappa-light chain deposits on the glomerular mesangium and capillary wall, tubules, and vessel wall. The electron microscopy demonstrated electron-dense deposits on the glomerular basement membrane and mesangium. Anemia was observed with schistocytosis and Howell-Jolly body in the peripheral blood smears. The immunohistochemical examination of the bone marrow showed the presence of kappa-light chain deposits in scattered plasma cells and thickened vessel wall in the absence of a prominent plasma cell proliferation. Although an immunofixation electrophoresis failed to detect a monoclonal gammopathy, the presence of monoclonal protein could be identified by an abnormal kappa/lambda ratio on the serum free light chain analysis.
Subject(s)
Female , Humans , Middle Aged , Anemia, Hemolytic/complications , Bone Marrow/pathology , Glomerulonephritis/complications , Immunoglobulin Light Chains/analysis , Kidney Glomerulus/pathology , Paraproteinemias/complicationsABSTRACT
We have had a recent spurt in cases of AIDS-related lymphoma (ARL) at our centre. Most of these cases are aggressive mature B cell lymphomas, mainly plasmablastic lymphoma (PBL) and diffuse large B-cell lymphoma (DLBCL). Most of the PBL are extranodal in location and are mucosa-based. We reviewed the morphological features of 34 cases of PBL. Diagnosis was based on morphology, immunohistochemistry, proliferation index, HIV positive status and its preference to extranodal sites (mostly mucosa based). We classified PBL into three morphological subtypes (immunoblastic - 25, Burkitt's - 7, plasmacytic - 2). Tumor cells expressed as leucocyte common antigen (LCA) in 60%, CD138 in 100%, EMA in 45% and light chain restriction in 86% cases. CD20 was negative in all cases. Pathologists need to be aware of PBL and its various morphological subtypes as the identification of this entity from its close differentials carries major therapeutic implications.
Subject(s)
Acquired Immunodeficiency Syndrome/complications , Adolescent , Adult , Aged , Antigens, CD20/analysis , Leukocyte Common Antigens/analysis , Burkitt Lymphoma/pathology , Child , Female , Humans , Immunoglobulin Light Chains/analysis , Leukemia, Plasma Cell/pathology , Lymphoma, AIDS-Related/pathology , Lymphoma, Large-Cell, Immunoblastic/pathology , Male , Middle Aged , Syndecan-1/analysisABSTRACT
La glándula tiroides puede presentar patologías que desde el punto de vista histológico muestran diferentes grados de infiltración linfocitaria y plasmocitaria. Con el fin de identificar los subtipos de células plasmáticas en algunas patologías tiroideas nosotros hemos realizado un estudio inmunohistoquímico sobre la presencia de cadenas pesadas (IgG, IgA, IgM) y livianas (K y L) que se pueden encontrar en el citoplasma de las células plasmáticas. En las tiroiditis de Hashimoto estudiadas se encontró un predominio de células plasmáticas conteniendo IgG y kappa. El estroma linoplasmocitario que a veces acompaña al carcinoma papilar de tiroides también mostró una mayor frecuencia de células plasmáticas con IgG, aunque lambda fue la cadena liviana predominante en este caso. Por otro lado, las tiroiditis de Riedel mostraron un mayor predominio de IgA y lambda. Los 6 casos de fibrosis retroperitoneal, enfermedad a veces asociada con tiroiditis de Riedel, mostraron en conjunto una distribución de subtipos de células plasmáticas similar a la observada en las tiroiditis de Riedel aunque la frecuencia de células con IgA fue menos marcada. Estos resultados sugieren que la desigual distribución de inmunoglobulinas citoplasmáticas en estas entidades estudiadas puede estar relacionada con la presencia de diferentes antígenos involucrados en el proceso patogénico que trae aparejado el infiltrado linfoplasmocitario