Glomerulonefritis proliferativa extracapilar pauciinmune, una manifestación paraneoplásica inusual asociada a linfoma B del manto

The relationship between neoplasia and secondary renal involvement is increasing. Membranous glomerulonephritis is the main cause of nephrotic syndrome, associated with solid tumors, and less frequently hematologic neoplasia. Hodgkin’s lymphoma is the hematologic neoplasia most closely associated with minimal change disease. However, there are case reports that describe a relationship with non-Hodgkin’s lymphomas and leukemias. The usual clinical manifestation is the nephrotic syndrome,which may precede for several months or coexist with the diagnosis of lymphoma. Its pathophysiology is not very clear. It is suggested that it is related to T lymphocyte dysfunction. In addition, there are reports of thrombotic microangiopathy, related to the use of biological therapy and antineoplastic agents.Rapidly progressive glomerulonephritis is a clinical syndrome associated with glomerular extracapillary proliferation, usually related to vasculitis associated with ANCA and vasculitis mediated by immunocomplexes. Neoplasias are also related to a large number of vasculopathies. Glomerulonephritis with extracapillary proliferation are much more common in malignant solid tumors. In addition, associations with myelodysplastic syndrome, chronic lymphocytic leukemia and Hodgking lymphoma have been reported. The association between Mantle Cell B lymphomas and glomerulonephritis with extracapillary proliferation is unusual. The following is a case of a patient diagnosed with Mantle Cell B lymphoma who has a rapidly progressive glomerulonephritis secondary to extracapillary, necrotizing and pauci-immune proliferative lesions.La relación entre neoplasia y compromiso renal secundario es creciente. La glomerulonefritis membranosa es la principal causa de síndrome nefrótico asociado a tumores sólidos y con menos frecuencia a neoplasia hematológica. El linfoma Hodgkin es la neoplasia hematológica que más se relaciona con enfermedad de cambios mínimos. Sin embargo, hay reportes de casos que describen una relación con linfomas no Hodgkin y leucemias. La manifestación clínica usual es el síndrome nefrótico; el cual puede preceder por varios meses o coexistir con el diagnóstico de linfoma. La fisiopatogénesis no está muy clara. Se sugiere que está relacionada con una disfunción del linfocito T. Adicionalmente, hay reportes de microangiopatía trombótica relacionada con el uso de terapia biológica y antineoplásicos.La glomerulonefritis rápidamente progresiva, es un síndrome clínico asociado a proliferación extracapilar glomerular; usualmente relacionado con vasculitis asociadas a ANCAS y vasculitis mediadas por inmunocomplejos. Las neoplasias también están relacionadas con un amplio número de vasculopatías. Las glomerulonefritis con proliferación extracapilar son mucho más frecuentes en tumores sólidos malignos.Además, se han descrito asociaciones con síndrome mielodisplásico, leucemia linfocítica crónica y linfoma Hodgkin. La asociación entre linfomas B del manto y glomerulonefritis con proliferación extracapilar, esinusual.A continuación, se describe un caso de un paciente con diagnóstico de linfoma B del manto que cursa con una glomerulonefritis rápidamente progresiva secundaria a lesiones proliferativas extracapilares, necrosantes y pauciinmunitaria

Glomerulonefritis membranoproliferativa secundaria a vasculitis crioglobulinémica mixta esencial

We present the case of a 48 years old man with no known comorbidities, who sought for medical atention at San Vicente Fundación Universitary Hospital, complaining of a two month history with progressive dyspnea, generalized edema and macroscopic urinary changes, in whom hypertension, progressive kidney function decline, azotemia, nephrotic syndrome (9 g/24 h proteinuria), active urinary sediment, hypocomplementemia (C3 and C4) and cryoglobulinemia were found on ancillary tests. The kidney ultrasonography did not reveal findings suggestive of chronicity and the electrophoresis/immunifixation tests showed a polyclonal hypergammaglobulinemic pattern (without light chains). On the kidney biopsy an immunocomplex mediated membranoproliferative glomerulonephritis pattern of lesion was found, with rheumatic and neoplastic etiologies being ruled out. The patient was diagnosed with idiopatic immunocomplex mediated membranoproliferative glomerulonephritis due to mixed essential cryoglobulinemic vasculitis and immunosuppressive therapy (for remission induction) was started, with resolution of edemas, recovery of kidney function and a progressive decline in proteinuria from 9gr/dl to less than 1.8gr/dl on follow up evaluation.Presentamos el caso de un paciente sin comorbilidad conocida quien consulta al HUSVF por cuadro clínico de 2 meses de evolución con deterioro progresivo de la clase funcional, edema generalizado asociado a cambios macroscópicos en la orina, documentándose hipertensión arterial, deterioro progresivo de la función renal, con hiperazoemia, sedimento urinario activo y proteinuria en 9 gramos con criterios de síndrome nefrótico, hipocomplementemia (C4 y C3), crioglobulinemia, electroforesis e inmunofijación sérica  y urinaria con patrón de hipergammaglobulinemia policlonal (sin cadenas livianas), ecografía renal sin hallazgos sugestivos de nefropatía crónica y hallazgos en biopsia renal compatibles con glomerulonefritis membranoproliferativa mediada por inmunocomplejos. Se descartaron las enfermedades reumatológicas, hematológicas e infecciosas implicadas en la etiología de esta entidad (HCV,HBV,HIV, entre otras causas) y bajo el diagnóstico de glomerulonefritis membranoproliferativa secundaria a vasculitis crioglobulinémica mixta esencial se administró terapia inmunosupresora para inducir remisión (en conjunto con manejo médico para sobrecarga hídrica), con resolución del cuadro de edemas, recuperación de la función renal y una reducción progresiva de la proteinuria.

Glomeruloesclerosis focal y segmentaria como manifestación paraneoplasica: A propósito de un caso.

Las glomerulonefritisparaneoplásicas son lesiones glomerulares que no están directamente relacionados con la carga tumoral o metástasis, Desde 1922 ha sido descrita la asociación de síndrome nefrótico(SN) con malignidad con una incidencia que Puede ser hasta de un 10%3, las “glomerulonefritis (GN) paraneoplásicas clásicas” son la GN membranosa asociada a tumor sólido y el linfoma Hodgkin con enfermedad de cambios mínimos. Describimos el casode un hombre que durante el estudio de un síndrome nefrótico se diagnosticóglomeruloesclerosis focal y segmentaria(GEFS) por biopsia renal, secundaria a Linfoma no Hodgkin B de bajo grado. Objetivo. Describir un caso clínico de una glomerulonefritisparaneoplásica cuya manifestación fue una asociación inusual

Glomerular diseases in a Hispanic population: review of a regional renal biopsy database

CONTEXT AND OBJECTIVE: Epidemiological data provide useful information for clinical practice and investigations. This study aimed to determine glomerular disease frequencies in a region of Colombia and it represents the basis for future studies. DESIGN AND SETTING: Single-center retrospective analysis at the University of Antioquia, Colombia. METHODS: All native renal biopsies (July 1998 to December 2007) were reviewed, but only glomerular diseases were analyzed. The diagnosis of each case was based on histological, immunopathological and clinical features. RESULTS: A total of 1,040 biopsies were included. In 302 cases (29.0%), the patient's age was < 15 years. Primary glomerular diseases were diagnosed in 828 biopsies (79.6%) and secondary in 212 (20.4%). The most common primary diseases were focal and segmental glomerulosclerosis (FSGS) (34.8%), immunoglobulin A (IgA) nephropathy (IgAN) (11.8%), membranous glomerulonephritis (MGN) (10.6%), minimal change disease (MCD) (10.6%), crescentic glomerulonephritis (GN) (5.6%), and non-IgA mesangial proliferative GN (5.6%). Postinfectious GN represented 10.7% of the diagnoses if included as primary GN. Lupus nephritis corresponded to 17.8% of the entire series. In adults, the order of the most frequent primary diseases was: FSGS, IgAN, MGN, crescentic GN and MCD. In children (< 15 years), the most frequent were: FSGS, postinfectious GN, MCD, non-IgA mesangial proliferative GN, endocapillary diffuse GN and IgAN. CONCLUSIONS: As among Afro-Americans, FSGS is the most frequent type of glomerulopathy in our population, but in our group, there are more cases of IgAN. The reasons for these findings are unclear. This information is an important contribution towards understanding the prevalence of renal diseases in Latin America