RESUMEN
We present a unique case of cryoglobulinemic glomerulonephritis associated with nodal and renal infiltration by T-cell lymphoma of T-follicular helper phenotype. The patient presented with transient neurologic symptoms, severe nephritic syndrome with nephrotic-range proteinuria, and acute kidney injury. He had elevated double-stranded DNA levels, low complement levels, detectable cryoglobulin, and detectable immunoglobulin M (IgM) paraprotein. The kidney biopsy showed cryoglobulinemic glomerulonephritis with a membranoproliferative pattern and diffuse interstitial infiltrates on light microscopy; IgM, C3 but weak IgG, C1q, and negative C4d staining on immunofluorescence; and deposits with organized substructures on electron microscopy. Positron emission tomography showed diffuse uptake in bilaterally enlarged kidneys and a localized group of lymph nodes. Subsequent lymph node biopsy revealed Epstein-Barr virus-negative nodal T-cell lymphoma, which was also proven in renal tissue. The association between T-cell lymphoma, autoantibodies, and cryoglobulinemia may represent a paraneoplastic phenomenon. His renal prognosis has been excellent, but overall prognosis and survival is dictated by the clinical course of T-cell lymphoma.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Crioglobulinemia/complicaciones , Glomerulonefritis Membranoproliferativa/patología , Neoplasias Renales/patología , Linfoma de Células T/patología , Linfocitos T Colaboradores-Inductores/patología , Adulto , Biopsia con Aguja , Médula Ósea/patología , Crioglobulinemia/diagnóstico , Ciclofosfamida/uso terapéutico , Progresión de la Enfermedad , Doxorrubicina/uso terapéutico , Estudios de Seguimiento , Glomerulonefritis Membranoproliferativa/complicaciones , Glomerulonefritis Membranoproliferativa/diagnóstico por imagen , Humanos , Inmunohistoquímica , Neoplasias Renales/diagnóstico por imagen , Neoplasias Renales/tratamiento farmacológico , Linfoma de Células T/complicaciones , Linfoma de Células T/diagnóstico por imagen , Linfoma de Células T/tratamiento farmacológico , Masculino , Fenotipo , Tomografía de Emisión de Positrones/métodos , Prednisona/uso terapéutico , Medición de Riesgo , Factores de Tiempo , Resultado del Tratamiento , Vincristina/uso terapéuticoAsunto(s)
Dolor Abdominal/etiología , Endoscopía/efectos adversos , Neoplasias de Cabeza y Cuello/patología , Carcinoma de Células Escamosas de Cabeza y Cuello/secundario , Neoplasias Gástricas/diagnóstico por imagen , Neoplasias Gástricas/secundario , Dolor Abdominal/diagnóstico por imagen , Gastrostomía/efectos adversos , Humanos , Masculino , Persona de Mediana Edad , Siembra NeoplásicaRESUMEN
Duplication cysts are rare benign congenital malformations typically identified in children by the age of 2 years. We report a rare case of colonic duplication cyst with dysplasia in an adult. A 32-year-old male was diagnosed with non-specific abdominal symptoms. Abdominopelvic computed tomography scan demonstrated a submucosal cystic lesion in the right colon. He underwent laparoscopic right hemicolectomy. Histopathology showed colonic duplication cyst with low grade dysplasia. He is due for a surveillance colonoscopy in 3 years. Duplication cyst in an adult colon with dysplasia is extremely rare. They are usually present in the terminal ileum. They have non-specific abdominal symptoms or can be asymptomatic. They are often identified incidentally or intraoperatively. Imaging may demonstrate a cystic lesion. Histopathology is required for definitive diagnosis. There are no guidelines or consensus on managing duplication cysts in adults. We recommend an oncological resection of the involved colon. Surveillance with routine colonoscopy is advisable.
Asunto(s)
Virus BK , Neoplasias Renales/diagnóstico , Nefritis Intersticial/diagnóstico , Infecciones por Polyomavirus/diagnóstico , Infecciones Tumorales por Virus/diagnóstico , Humanos , Pruebas de Función Renal , Neoplasias Renales/complicaciones , Neoplasias Renales/fisiopatología , Neoplasias Renales/virología , Leucemia Linfocítica Crónica de Células B/complicaciones , Leucemia Linfocítica Crónica de Células B/fisiopatología , Linfocitos Infiltrantes de Tumor , Masculino , Persona de Mediana Edad , Nefritis Intersticial/complicaciones , Nefritis Intersticial/fisiopatología , Infecciones por Polyomavirus/complicaciones , Infecciones por Polyomavirus/fisiopatología , Infecciones Tumorales por Virus/complicaciones , Infecciones Tumorales por Virus/fisiopatologíaRESUMEN
Granular cell tumours (GCTs) are generally benign neoplasms, which are believed to be of neural origin. They are uncommon in the gastrointestinal tract. They are rarely found in the colon and even more rarely found to be multiple. We present a case of a man who underwent a right hemicolectomy for a submucosal lesion and polyps and was found to have multiple nodules diagnosed as caecal GCTs with cellular atypia. While uncommon, this case shows it remains an important differential due to implications for patient management, given the often benign nature of disease.
RESUMEN
Background: cDC1 is a subset of conventional DCs, whose most recognized function is cross-presentation to CD8+ T cells. We conducted this study to investigate the number and location of cDC1s in various human kidney diseases as well as their correlation with clinico-pathological features and CD8+ T cells. Methods: We analyzed 135 kidney biopsies samples. Kidney diseases included: acute tubular necrosis (ATN), acute interstitial nephritis (AIN), proliferative glomerulonephritis (GN) (IgA nephropathy, lupus nephritis, pauci-immune GN, anti-GBM disease), non-proliferative GN (minimal change disease, membranous nephropathy) and diabetic nephropathy. Indirect immunofluorescence staining was used to quantify cDC1s, CD1c+ DCs, and CD8+ T cells. Results: cDC1s were rarely present in normal kidneys. Their number increased significantly in ATN and proliferative GN, proportionally much more than CD1c+ DCs. cDC1s were mainly found in the interstitium, except in lupus nephritis, pauci-immune GN and anti-GBM disease, where they were prominent in glomeruli and peri-glomerular regions. The number of cDC1s correlated with disease severity in ATN, number of crescents in pauci-immune GN, interstitial fibrosis in IgA nephropathy and lupus nephritis, as well as prognosis in IgA nephropathy. The number of CD8+ T cells also increased significantly in these conditions and cDC1 number correlated with CD8+ T cell number in lupus nephritis and pauci-immune GN, with many of them closely co-localized. Conclusions: cDC1 number correlated with various clinic-pathological features and prognosis reflecting a possible role in these conditions. Their association with CD8+ T cells suggests a combined mechanism in keeping with the results in animal models.
Asunto(s)
Linfocitos T CD8-positivos/inmunología , Reactividad Cruzada , Células Dendríticas/inmunología , Enfermedades Renales/inmunología , Riñón/inmunología , Adulto , Anciano , Biopsia , Estudios de Casos y Controles , Femenino , Fibrosis , Técnica del Anticuerpo Fluorescente , Humanos , Riñón/patología , Enfermedades Renales/metabolismo , Masculino , Persona de Mediana Edad , Fenotipo , PronósticoRESUMEN
We present 6 cases with multifocal appendiceal neuroendocrine tumors, including their clinical and histopathological findings. To our knowledge, this is the first description of a multifocal pattern of a neuroendocrine neoplasm arising in the appendix. All patients presented in a setting requiring an acute appendectomy. The number of tumors ranged from 2 to 5. Histopathological examination revealed WHO (World Health Organization) grade 1 tumor in 3 patients and WHO grade 2 in the other 3 patients. The median duration of follow-up in these patients was 70 months (range = 6-192 months). No metastatic disease was observed. According to these findings, a multifocal pattern of neuroendocrine neoplasms along the appendix seems not to be a predictor for local advanced or metastatic disease.