Ileítis como presentación de linfoma en síndrome de Wiskott-Aldrich / Ileitis as presentation of lymphoma in Wiskott-Aldrich syndrome

El síndrome de Wiskott-Aldrich (SWA) es un raro síndrome de inmunodeficiencia primaria ligado al cromosoma X que se asocia con aumento de incidencia de infecciones, trastornos autoinmunes y neoplasias. Se presenta el caso de un varón de 41 años con diagnóstico de síndrome de Wiskott-Aldrich y cuadro de ileítis como forma de presentación de un síndrome linfoproliferativo. La ileítis, en el contexto del paciente, representa un problema clínico dado el gran número de diagnósticos diferenciales (enfermedad inflamatoria intestinal, infecciones, neoplasias y enfermedades linfoproliferativas) por lo que suele requerir diagnóstico anatomopatológico y consideraciones particulares respecto al posterior tratamiento específico.

Wiskott-Aldrich syndrome is a rare X chromosome-linked primary immunodeficiency syndrome associated with an increased incidence of infections, autoimmune disorders and neoplasms. We present the case of a 41-year-old man with a diagnosis of Wiskott-Aldrich syndrome with ileitis as a form of presentation of a lymphoproliferative syndrome. The ileitis, in the context of the patient, represents a clinical challenge given the large number of differential diagnoses (inflammatory bowel disease, infections, neoplasms and lymphoproliferative diseases), so it usually requires anatomopathological diagnosis and particular considerations regarding the subsequent specific treatment.

Coexistencia de colitis linfocítica y enteritis microscópica en pacientes con diarrea crónica / Concurrent lymphocytic colitis and microscopic enteritis in patients with chronic diarrhea

Introducción: Colitis linfocítica y enteritis microscópica son causas relativamente comunes de diarrea crónica y ambas se caracterizan por un infiltrado linfocitico intraepitelial. No existen reportes previos de la coexistencia de ambas entidades. Objetivo: Describir las características clínicas e histológicas de los pacientes que presentan este diagnóstico simultáneamente. Material y métodos: Se seleccionaron pacientes adultos con diarrea crónica que tuvieran biopsia simultánea de colon y duodeno tomados el mismo día, durante los años 2010-2016, en el Servicio de Gastroenterología del Hospital Nacional Daniel Alcides Carrión. Se recopiló información clínica del archivo de historias. Las láminas fueron reevaluadas histológicamente por 3 patólogos. Se realizó estudio inmunohistoquímico de linfocitos intraepiteliales para CD8 y CD3 en 6 casos. Resultados: De 63 pacientes con diarrea crónica y biopsia simultánea de duodeno y colon, se identificó un total de 35 pacientes (55,5%) con diagnóstico simultáneo de enteritis microscópica y colitis linfocítica, 80% fueron mujeres. Se identificó anemia en 28,5% de los pacientes e infestación por Blastocystis hominis en el 31,8.%. En enteritis microscópica, el promedio de linfocitos intraepiteliales con CD8 y CD3 fue 40%, mientras que, en colitis linfocítica, el promedio fue de 37,2% para CD3 y 29,2% para CD8. En 11 de los 35 casos, se pudo obtener biopsias de íleon que fueron diagnosticadas como ileitis linfocítica. En 9 casos se diagnosticó colitis eosinofílica asociada a colitis linfocítica. Conclusión: Se encontró coexistencia de colitis linfocítica, enteritis microscópica y en algunos de ileitis linfocítica en un 55,5% pacientes con diarrea crónica con biopsia duodenal y colónica. Estos resultados abren la interrogante sobre si colitis linfocítica y enteritis microscópica son entidades diferentes o constituyen una sola patología que en algunos pacientes afecta varios segmentos del tubo digestivo.

Introduction: Lymphocytic colitis and microscopic enteritis are relatively common causes of chronic diarrhea and it is characterized by an intraepithelial lymphocytic infiltrate. There have been no previous reports of coexistence between these 2 pathologies. Objective: To describe histological and clinical characteristic in patients with coexistence of lymphocytic colitis and microscopic enteritis. Material and methods: All cases with simultaneous diagnosis of lymphocytic duodenosis and lymphocytic colitis were reevaluated during lapse time 2010-2016 in hospital Daniel Carrion. The slides were reviewed by 3 pathologists and clinical information was obtained from clinical records. Expression of CD3 and CD8 was detected in 6 cases by immunohistochemical assays. Results: A total of 35 patients with coexistence of lymphocytic duodenitis and lymphocytic colitis were selected of the pathology archives, 80% were females, Anemia was identified in 28.5% of patients. Blastocysitis hominis infestation was identified in 31.8%. The mean intraepithelial lymphocyte CD8 and CD3 positive was 40% in microscopic enteritis, while the mean intraepithelial lymphocyte CD3 positive was 37.2% and CD8 positive was 29.2% Additionally, lymphocytic ileitis was diagnosed in 11 of our cases. Eosinophilic colitis was diagnosed in 9 cases of lymphocytic colitis Conclusion: We found that lymphocytic colitis, microscopic enteritis and even lymphocytic ileitis can coexist in a group of patients with chronic diarrhea. These findings bring the question if this concurrence of both pathologies constituted a more generalized gastrointestinal disorder, involving both the large and the small intestines.

Evaluation of the immunoexpression of COX-1, COX-2 and p53 in Crohn's disease / Avaliação da imunoexpressão de COX-1, COX-2 e p53 na doença de Crohn

BACKGROUND: Crohn's disease accompanied by nonspecific or idiopathic ulcerative proctocolitis corresponds to a condition called intestinal inflammatory disease. The immunoexpression of cyclooxygenase 2 (COX-2) in Crohn's disease becomes more marked with progression of the disease and the presence of wild-type p53 suppresses the transcription of COX-2. AIMS: To investigate the immunoexpression of cyclooxygenase 1 (COX-1), COX-2 and p53 in Crohn's ileocolitis and to correlated this expression with clinical and histopathological parameters. METHODS: Forty-five cases of Crohn's disease, 16 cases of actinic colitis (diseased-control group) and 11 cases without a history of intestinal disease (normal control group) were studied. Hematoxylin-eosin-stained sections were submitted to histopathological analysis and the immunohistochemical expression of COX-1, COX-2 and p53 was evaluated by the streptavidin-biotin-peroxidase method. RESULTS: Sixty percent of the Crohn's disease patients were women and 40 percent were men, with 75.5 percent whites and 25.5 percent non-whites. The disease involved the terminal ileum in 44.5 percent of cases, ileum in 33.3 percent, colon in 20 percent and duodenum-ileum in 2.2 percent. A significant association was observed between COX-2 immunoreactivity and age <40 years. Histopathological analysis of Crohn's disease samples showed mild or moderate crypt distortion (57.8 percent and 35.6 percent of cases), atrophy (6.6 percent), mild, moderate and marked chronic inflammation (46.7 percent, 26.7 percent and 20 percent), acute inflammatory activity (93.3 percent), ulceration (24.4 percent), mucin depletion (37.8 percent), Paneth's cells (24.4 percent), intraepithelial lymphocytes (93.3 percent), and subepithelial collagen (6.7 percent). In the CD group, COX-1 immunoreactivity in epithelial and inflammatory cells was observed in 26.7 percent and 22.2 percent of cases, respectively. COX-2 immunoreactivity was detected...

RACIONAL: A doença de Crohn, junto com a colite ulcerativa idiopática ou inespecífica constituem a doença inflamatória intestinal. A imunoexpressão de ciclooxigenase 2 (COX-2) na doença de Crohn acentua-se com a progressão da doença, enquanto que a presença do tipo selvagem de p53 suprime a transcrição de COX-2. OBJETIVOS: Investigar a imunoexpressão de ciclooxigenase 1 (COX-1), COX-2 e p53 na doença de Crohn e correlacionar os achados com parâmetros clínico-histopatológicos. MÉTODOS: Foram estudados 45 casos de doença de Crohn (grupo teste), 16 casos de colite actínica (grupo controle-doente) e 11 casos sem história de doença intestinal (grupo controle normal). A avaliação histopatológica foi feita com lâminas coradas pela hematoxilina-eosina e a imunoexpressão de COX-1, COX-2 e p53 foi avaliada por imunoistoquímica, pelo método da estrepto-avidina-biotina-peroxidase. RESULTADOS: Entre os pacientes com doença de Crohn, 60 por cento eram do sexo feminino e 40 por cento do masculino, 75,5 por cento brancos e 25,5 por cento não-brancos. A doença comprometia o íleo terminal em 44,5 por cento dos casos, íleo em 33,3 por cento, cólon em 20 por cento e duodeno-íleo em 2,2 por cento. Associação significante foi detectada entre a imunoexpressão de COX-2 e pacientes com <40 anos. A histopatologia dos casos de doença de Crohn mostrou distorção críptica em grau leve ou moderado (57,8 por cento e 35,6 por cento dos casos), atrofia (6,6 por cento), inflamação focal, difusa superficial e difusa transmural (46,7 por cento, 26,7 por cento e 20 por cento), inflamação aguda neutrofílica (93,3 por cento), alterações epiteliais: ulceração (24,4 por cento), depleção de mucina (37,8 por cento), células de Paneth (24,4 por cento); alterações epiteliais associadas: linfócitos intra-epiteliais (93,3 por cento) e colágeno subepitelial (6,7 por cento). No grupo doença de Crohn, imunoexpressão de COX-1, em células epiteliais e inflamatórias foi observada em 26,7...

Non specific jejunoileitis--a report of 8 cases.

Nonspecific jejuno-ileitis is a nonocclusive, necrotizing inflammation of the small intestine. We treated 8 patients of jejuno-ileitis in a short span of 8 months. Their mean age was 8.6 years. All had acute pain in abdomen and most had hematochezia. Radiology was helpful only in diagnosis of complications of the disease. Four patients responded to conservative management; the other 4 required surgery--laparotomy and lavage in 2, and multiple laparotomies with resections in 2. One patient died due to chronic malnutrition and metabolic complications. Bowel histology was suggestive of resolving vasculitis in one patient and chronic inflammation in another patient.

Historia de las manifestaciones gastrointestinales del lupus eritematoso sistemico / History of the gastrointestinal manifestations in systemic lupus erythematosus

En este articulo revisamos la historia de las manifestaciones gastrointestinales en el Lupus Eritematoso Sistemico desde el siglo XIX hasta nuestros dias, recorriendo cada uno de los organos involucrados en este sistema y haciendo especial mencion de la gastropatia, enteritis, ileitis, sindrome de malabsorcion, vasculitis y vasculopatia intestinal, trombosis mesenterica, pancreatitis, ascitis, peritonitis, hepatitis autoinmune, entre otros

Crohn's disease: a case report

A case of rather typical Crohn's disease in a 10 year old girl is described. She had suffered from intractable abdominal pain, diarrhea and fever for 1 year. Eventual right hemicolectomy revealed diffuse involvement of terminal ileum, cecum and ascending colon by confluent ulcerations and transmural inflammation. Histologically there were numerous well developed non-caseating granulomas scattered transmurally and in regional lymph nodes. Deep penetrating ulcerations were characteristic. Acid fast staining failed to demonstrate any organism. The rarity of Crohn's disease in Korea and this occurrence in pediatric age prompted this report.