Intraoral primary syphilis mimicking lymphoproliferative disorder.

Syphilis can mimic, clinically and microscopically, many other diseases. By microscopy, typically syphilis presents with plasma cell infiltration, admixed with lymphocytes and macrophages, in lichenoid and/or perivascular/perineural distribution pattern. When exuberant, this inflammatory infiltrate can mimic a lymphoproliferative disorder (LPD), notably plasma cell neoplasia or lymphoma. To date, about 12 cases of secondary syphilis, all but one in extraoral location, suggesting initially a LPD, have been published. Here, to our knowledge, we report an unusual case of intraoral primary syphilis initially suggesting LPD, notably lymphoid hyperplasia (pseudolymphoma); however, mucosa-associated lymphoid tissue (MALT) lymphoma and follicular lymphoma could not be disregarded. Polyclonality of plasma cells on immunohistochemistry, in strict clinical correlation, was essential to arrive at the correct diagnosis.

Validez del hallazgo de nódulos en la colonoscopia para el diagnóstico de hiperplasia nodular linfoide en niños / Validity of nodules detected at colonoscopy for the diagnosis of nodular lymphoid hyperplasia in children

Resumen Introducción: la hiperplasia nodular linfoide del colon se define como > 10 nódulos linfoides visibles en colonoscopia. No existen estudios de su validez al compararlo con la histopatología. Objetivos: determinar la validez del hallazgo de nódulos en colonoscopia para el diagnóstico de hiperplasia nodular linfoide. Material y métodos: estudio prospectivo de prueba diagnóstica. Se incluyeron colonoscopias realizadas consecutivamente de 2014 al 2018 con equipos Olympus PCFQ150AI y GIFXP150N con obtención de biopsias. El criterio endoscópico fue la presencia de > 10 nódulos de 2 a 10 mm y el criterio histológico fue hiperplasia de folículos linfoides y mantos de linfocitos en lámina propia o submucosa. Los datos se analizaron en Epidat3.1. Se obtuvo la sensibilidad (S), especificidad (E), valor predictivo positivo (VPP) y negativo (VPN), y coeficientes de probabilidad positivo (LR+) y negativo (LR-) con sus intervalos de confianza. Resultados: se incluyeron 327 colonoscopias, la mediana de edad fue de 84 meses. La principal indicación para la colonoscopia fue sangrado digestivo bajo (38,8%). El hallazgo de nódulos se encontró en el 21% y el sitio de mayor frecuencia fue el colon total (46%), mientras que por histopatología se encontró hiperplasia nodular linfoide en el 38%. El hallazgo de nódulos obtuvo una S de 32% (intervalo de confianza [IC] del 95%: 24-140), E de 84% (IC 95%: 79-89), VPP de 56% (IC 95%: 44-68), VPN de 67% (IC 95%: 61-72), LR+ de 2,04 (IC 95%: 1,4-3) y LR- de 0,8 (IC 95%: 0,8-0,9). Conclusiones: la validez diagnóstica del hallazgo de nódulos en colonoscopia para hiperplasia nodular linfoide es pobre, por lo que la toma de biopsia debe recomendarse siempre.

Abstract Introduction: Nodular lymphoid hyperplasia of the colon is characterized by the presence of >10 lymphoid nodules visible in colonoscopy. There are no studies that confirm their validity when compared with histopathology. Objective: To determine the validity of nodules detected at colonoscopy for the diagnosis of nodular lymphoid hyperplasia in children. Materials and methods: Prospective study of diagnostic test accuracy. Colonoscopies performed consecutively from 2014 to 2018 using Olympus PCFQ150AI and GIFXP150N biopsy machines were included. The endoscopic criterion was the presence of >10 nodules from 2 to 10mm of diameter, while the histological criterion was presence of follicular lymphoid hyperplasia and lymphocyte mantles in lamina propia or submucosa. Data were analyzed in Epidat3.1. Sensitivity (SE), specificity (SP), positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio (LR+) and negative likelihood ratio (LR-) were obtained with their corresponding confidence intervals. Results: 327 colonoscopies were included; the median age was 84 months. The main indication for colonoscopy was lower gastrointestinal bleeding (38.8%). Nodules were found in 21% of the patients, predominantly throughout the whole colon (46%), whereas histopathology found nodular lymphoid hyperplasia in 38%. SE for the finding of nodules was 32% (95% confidence interval [CI]: 24-140), SP was 84% (95% CI: 79-89), PPV was 56% (95% CI: 44-68), NPV was 67% (95% CI: 61-72), LR+ was 2.04 (95% CI: 1.4-3) and LR- was 0.8 (95% CI: 0.8-0.9). Conclusions: The validity of the presence of nodules on colonoscopy for the diagnosis of nodular lymphoid hyperplasia is poor, so biopsy should always be performed.

Oral follicular lymphoid hyperplasia: clinicopathologic of a case series / Hiperplasia linfoide folicular oral: clinicopatológico de uma série de casos

RESUMEN La hiperplasia folicular linfoide (HFL) es una proliferación linfoide reactiva que puede simular linfomas, tanto clínica como histológicamente. El objetivo de este estudio fue investigar las características clínicas, morfológicas e inmunohistoquímicas de una serie de casos de HFL en la cavidad oral y discutir importantes aspectos diagnósticos y diagnósticos diferenciales en relación con los linfomas foliculares. Un análisis retrospectivo de los registros de una base de datos de 38 años reveló nueve casos diagnosticados como HFL de la cavidad oral. La edad de los pacientes osciló entre 8 y 44 años. La mayoría de las lesiones se localizaron en la mucosa oral y la presencia de un nódulo indoloro fue el hallazgo clínico más común. El análisis histopatológico reveló proliferación de células linfoides dispuestas en patrón folicular, presentando folículos primarios y secundarios con centro germinal y zona del manto, con evidencia de macrófagos que contenían cuerpos apoptóticos en su interior, así como evidencia de figuras de mitosis típicas. Observamos el área interfolicular, los linfocitos, los macrófagos e las islas epimioepiteliales. El análisis inmunohistoquímico reveló positividad de folículos linfoides para CD20, CD68, CD3 y linfoma de células B2 (Bcl-2). La presentación clínica de HFL y las evidencias histopatológicas de folículos linfáticos que muestran centros germinales indistintos con una zona del manto mal definida pueden ser un problema debido a la similitud con el linfoma folicular.

Estudios de laboratorio como herramienta en la identificación de apendicitis aguda y su diferenciación con hiperplasia linfoide / Laboratory studies as a tool for the differential diagnosis between acute appendicitis and lymphoid hyperplasia

RESUMEN Antecedentes: La apendicitis aguda (AA) es una de las principales patologías quirúrgicas en México y el mundo. A pesar de ser una patología frecuente, el manejo quirúrgico presenta una tasa del 10-20% de apendicitis blancas (AB) o hiperplasia folicular linfoide (HPL) por histopatología. Objetivo: Comparar los hallazgos clínicos, de laboratorio y radiológicos de pacientes con sospecha de AA con su diagnóstico por histopatología. Material y métodos: El estudio es retrospectivo, observacional y transversal. Se analizaron los datos de los pacientes con diagnóstico clínico de apendicitis aguda, desde febrero de 2013 hasta diciembre de 2017, atendidos en un hospital de tercer nivel en Monterrey, México. Se compararon los hallazgos de los pacientes con diagnóstico de AA vs. HPL, AA no complicadas vs. AA complicadas y pacientes pediátricos vs. adultos. Se realizó un análisis descriptivo por medio de frecuencias y porcentajes, y mediana y rango intercuartil (RIC), dada la distribución no paramétrica de estas variables. Se compararon los hallazgos por laboratorio mediante la prueba de Mann-Whitney. Se consideró una p< 0,05 como estadísticamente significativa. Resultados: En nuestro medio, ambos sexos tienen la misma frecuencia de presentación de AA. El tiempo de evolución es significativo en la presentación frecuente de AA complicada. Existe asociación entre leucocitosis, neutrofilia total y porcentual y recuento plaquetario mayor en presencia de AA vs. HPL. Conclusión: Los estudios de laboratorio no muestran cambios significativos en pacientes pediátricos con AA. En adultos con AA no complicada vs. apendicitis complicada, el aumento en WBC, NEU y NEU% son estadísticamente significativos.

ABSTRACT Background: Acute appendicitis (AA) is one of the main surgical pathologies in our country and worldwide. Despite being a surgery that is frequently done, it is still reported a 10-20% of negative appendectomies (NA). Objective: The objective of the study is to compare clinical, laboratory and radiology results with the histopathology diagnosis. Material and methods: A retrospective study was done analyzing the data of patients with clinical diagnosis of AA from February 2013 to December 2017, in a tertiary hospital in Monterrey, Mexico. They were classified by their histopathological results into different groups: AA or NA; and the AA was subdivided into complicated AA and uncomplicated AA. Finally, these groups were also subdivided by ages, into pediatric and adult groups of each category. A descriptive analysis was made using frequencies, percentages, median and the interquartile range. Laboratory results were compared with the Mann-Whitney test. Considering a p-value of p < 0.05 as statistically significant. Results: In our group of patients both genders had AA in a similar frequency, the time between the appearance of symptoms and reaching for medical advice was an important factor for having complicated AA. There is a correlation between leukocytosis, neutrophil count and platelet count elevated in presence of AA against NA. Conclusion: Laboratory studies did not report significant changes in pediatric patients with AA. In adults with uncomplicated AA vs. complicated AA, white blood cell count, and neutrophil count are statistically significant.

Diferenciar la apendicitis con o sin fecalito ¿puede cambiar el enfoqueterapéutico?: Resultados preliminares / Differentiating between appendicitis with or without fecalith to change the therapeutic approach: Preliminary results

Antecedentes: el manejo de los pacientes con apendicitis aguda fue clásicamente quirúrgico. En la era de los antibióticos se plantean nuevos paradigmas terapéuticos. La diferencia en el origen de la apendicitis podría establecer el tratamiento por elegir. Objetivo: analizar si la presencia de fecalito en una apendicitis puede condicionar un tratamiento quirúrgico o solo con antibióticos. Material y métodos: análisis descriptivo observacional de las diferencias intraoperatorias y anatomopatológicas de los pacientes operados por apendicitis aguda, divididos en 2 grupos según presentaran fecalito o hiperplasia linfoidea como causa de origen. Se utilizó la prueba de Chi2 para la comparación de ambos grupos, tomando un valor de p<0,05. Resultados: el grupo de apendicitis por fecalito presentó un estadio más avanzado de la enfermedad, con líquido libre con más frecuencia (el 67% de los pacientes con apendicitis aguda por fecalito vs. el 18% en el grupo de apendicitis aguda por hiperplasia), en más de una localización (solo el grupo con fecalito presentó líquido en el fondo de saco de Douglas o en el resto del abdomen, en el 50% y 16,7% respectivamente), con características que variaron entre seroso y purulento y anatomopatológicamente presentaron mayor afectación de las capas histológicas (en el grupo hiperplasia la afectación hasta la mucosa fue 63,6% vs. 16,7%, mientras que en el grupo fecalito fue más frecuente la afectación hasta la serosa 66,6% vs. 27,3%). Conclusiones: los pacientes con apendicitis aguda por hiperplasia linfoidea tendrían menos complicaciones intraabdominales en el posoperatorio y podrían ser buenos candidatos a tratamiento médico solo con antibióticos, evitando la cirugía.

Background: Surgery is the traditional approach for patients with acute appendicitis. In the antibiotic era, new therapeutic paradigms are being proposed. The difference in the cause of appendicitis could establish the treatment of choice. Objective: to analyze if the presence of fecalith in an appendicitis can condition a surgical treatment or only with antibiotics. Material and methods: This observational analysis describes the intraoperative and pathological differences between patients undergoing surgery for acute appendicitis, divided into two groups according to the presence of fecalith or lymphoid hyperplasia as cause of the condition. The chi square test was used to compare the fecalith group versus the lymphoid hyperplasia group using a p value < 0.05. Results: The presence of fecaliths was more commonly associated with advanced stage of inflammation, presence of free peritoneal fluid (67% vs. 18% in the lymphoid hyperlasia group) and in more than one site (50% in the Douglas' pouch and 16.7% in the rest of the abdominal cavity). In this group, peritoneal fluid varied between serous and purulent and more appendiceal layers were involved (63.6% of mucous layer involvement in the lymphoid hyperplasia group vs. 16.7%, while serous compromise was more common in the fecalith group: 66.6% vs. 27.3%). Conclusions: Patients with acute appendicitis due to lymphoid hyperplasia could have lower rate of post-operative complications or could be good candidates for medical treatment with antibiotics alone, avoiding surgery.

Hiperplasia linfoide ileo-colónica en pacientes pediátricos / Ileo-colonic lymphoid hyperplasia in pediatric patients

Introducción: La Hiperplasia nodular linfoide gastrointestinal constituye una entidad infrecuente con manifestaciones clínicas diversas y con mayor frecuencia en la edad pediátrica. Objetivo: Caracterizar clínica, endoscópica e histológicamente a los pacientes pediátricos con hiperplasia linfoide de colon e íleon terminal diagnosticados en el Instituto de Gastroenterología. Material y Métodos: Se realizó un estudio descriptivo, transversal, en el periodo comprendido entre 2014 y 2016 en el Instituto de Gastroenterología. La muestra estuvo constituida por 50 pacientes, quienes cumplieron los criterios de inclusión y exclusión. Se evaluaron variables demográficas, clínicas, endoscópicas e histológicas, así como el comportamiento de la comorbilidad con enfermedades malignas, enfermedades inflamatorias intestinales, giardiosis, trastornos de la respuesta inmunohumoral y alergias alimentarias. Resultados: El sexo masculino, entre 7-10 años y el color de la piel blanca fueron los más frecuentes. El sangrado rectal fue el síntoma principal (62 por ciento) y la localización a nivel del íleon terminal en 69 por ciento, no se relacionó con enfermedad maligna, hubo tres pacientes con diagnóstico de hiperplasia linfoide de íleon terminal y colitis ulcerosa. El 74 por ciento presentó aspecto nodular por histología y 60 por ciento se le diagnosticó Giardia lamblia, en la evaluación inmunohumoral predominó los pacientes sin inmunocompromiso (78 por ciento), el Prick Test fue positivo en 60 por ciento, sobre todo, a la leche de vaca. Conclusiones: La manifestación clínica que predominó fue el sangrado rectal, endoscópicamente la localización en íleon y la forma nodular por histología. No encontramos comorbilidades con enfermedades malignas y fueron más frecuentes los trastornos alérgicos y parasitarios que las alteraciones inmunológicas(AU)

Introduction: Lymphoid nodular hyperplasia of the gastrointestinal tract is an uncommon entity with diverse clinical manifestations, which is more frequent in the pediatric age. Objective: To characterize clinically, endoscopically, and histologically, those pediatric patients with lymphoid hyperplasia of the colon and terminal ileum diagnosed in the Institute of Gastroenterology. Material and Methods: A descriptive, cross-sectional study was carried out during the period between 2014 and 2016 in the Institute of Gastroenterology. The sample consisted of 50 patients who met the inclusion and exclusion criteria. Demographic, clinical, endoscopic and histological variables were evaluated, as well as the behavior of comorbidity with malignant diseases, inflammatory bowel diseases, giardiasis, disorders of the humoral immune response and food allergies. Results: The male sex, the age group between 7-10 years, and the white skin color were the most frequent. Rectal bleeding was the main symptom (62 percent), and the location of the lesions in the terminal ileum was observed in 69 percent of the patients. There was no relationship between lymphoid hyperplasia and malignant disease, but three patients were diagnosed with lymphoid hyperplasia of the terminal ileum, and ulcerative colitis. 74 percent of the biopsies presented a nodular variety, and 60 percent of the patients were diagnosed with Giardia lamblia; the cases that were not immunocompromised prevailed in the evaluation of humoral immune response (78 percent); the Prick Test was positive in 60 percent of children, especially to cow's milk. Conclusions: The predominant clinical manifestation was rectal bleeding; the localization was in the ileum, which was seen by endoscopic procedure; and the nodular form was demonstrated by histology. We did not find comorbidities with malignant diseases, and allergic and parasitic disorders were more frequently diagnosed than immunological alterations(AU)

Study of the allergenic potential of Bacillus thuringiensis Cry1Ac toxin following intra-gastric administration in a murine model of food-allergy.

Cry1Ac toxin, from Bacillus thuringiensis, is widely used as a biopesticide and expressed in genetically modified (GM) plants used for human and animal consumption. Since Cry1Ac is also immunogenic and able to activate macrophages, it is crucial to thoroughly evaluate the immunological effects elicited after intra-gastric administration. The allergenic potential of purified Cry1Ac was assessed and compared with that induced in a murine model of food-allergy to ovalbumin (OVA), in which animals are sensitized with the adjuvant Cholera toxin (CT). Mice were weekly intragastrically administered with: i) vehicle phosphate-buffered saline (PBS), ii) OVA, iii) OVA plus CT iv) Cry1Ac or v) OVA plus Cry1Ac. Seven weeks after, mice were intragastrically challenged and allergic reactions along with diverse allergy related immunological parameters were evaluated at systemic and intestinal level. The groups immunized with, Cry1Ac, OVA/Cry1Ac or OVA/CT developed moderate allergic reactions, induced significant IgE response and increased frequencies of intestinal granulocytes, IgE+ eosinophils and IgE+ lymphocytes. These same groups also showed colonic lymphoid hyperplasia, notably in humans, this has been associated with food allergy and intestinal inflammation. Although the adjuvant and allergenic potential of CT were higher than the effects of Cry1Ac, the results show that applied intra-gastrically at 50 µg doses, Cry1Ac is immunogenic, moderately allergenic and able to provoke intestinal lymphoid hyperplasia. Moreover, Cry1Ac is also able to induce anaphylaxis, since when mice were intragastrically sensitized with increasing doses of Cry1Ac, with every dose tested, a significant drop in rectal temperature was recorded after intravenous challenge.

Rare case report of an aggressive follicular lymphoid hyperplasia in maxilla.

Follicular lymphoid hyperplasia is a very rare though benign reactive process of an unknown pathogenesis that may resemble a follicular lymphoma, clinically and histologically. Oral reactive follicular hyperplasia (RFH) has been described on the hard or soft palate and at the base of the tongue. We describe here the first case of RFH presenting as an aggressive tumor on the right posterior side of the maxilla in a 24-year-old male patient. The lesion had a clinical evolution of 18 months and was noticed after the surgical extraction of the right third molar, although we cannot assume a cause-effect relation with that surgical event whatsoever. His medical history was unremarkable. Following an incisional biopsy, histological examination revealed lymphoid follicles comprised by germinal centers surrounded by well-defined mantle zones. The germinal centers were positive for Bcl-6, CD10, CD20, CD21, CD23, CD79a, and Ki-67, while negative for Bcl-2, CD2, CD3, CD5, and CD138. The mantle and interfollicular zones were positive for Bcl-2, CD2, CD3, CD5, CD20, and CD138. Both areas were diffusively positive for kappa and lambda, showing polyclonality. The patient underwent a vigorous curettage of the lesion with no reoccurrences at 36 months of follow-up. This case report demonstrates that morphologic and immunohistochemical analyses are crucial to differentiate RFH from follicular lymphoma, leading to proper management.

Conjunctival Tumors: Review of Clinical Features, Risks, Biomarkers, and Outcomes--The 2017 J. Donald M. Gass Lecture.

Conjunctival tumors encompass a broad range of diagnoses. The 3 most important malignant tumors include ocular surface squamous neoplasia (OSSN) (14%), melanoma (12%), and lymphoma (7%). Conjunctival malignancies are rarely found in children. Regarding OSSN, pre-disposing conditions include chronic solar radiation, immune deficiency (HIV), organ transplant, autoimmune conditions, xeroderma pigmentosum, and chronic exposure to cigarette smoke. OSSN is managed surgically or with topical/injection immunotherapy or chemotherapy. Metastasis occurs in <1%. Regarding melanoma, predisposing conditions include primary acquired melanosis (PAM), chronic nevus, and chronic solar radiation. Treatment of PAM or nevus can prevent melanoma. Melanoma management involves surgical resection with clean margins and avoidance of direct tumor manipulation ("no touch" technique). The first surgery is most important, to minimize tumor seeding. Biomarkers including BRAF, TERT, and PTEN provide information regarding risk for metastasis and allow for targeted antibiomarker therapies. Ten-year risk for melanoma metastasis is 25%. Tumors >2 mm thickness or those located in fornix, caruncle, or orbit are at highest risk for metastasis. Regarding lymphoma, predisposing conditions include benign reactive lymphoid hyperplasia, immune deficiency (HIV), immune dysfunction, and chronic inflammation/infection (Helicobacter pylori, Chlamydia psittaci). The 4 most important subtypes include extranodal marginal zone lymphoma (ENMZL), follicular lymphoma, mantle cell lymphoma (MCL), and diffuse large B-cell lymphoma. Treatment includes surgical resection, cryotherapy, radiotherapy, systemic chemotherapy, or targeted anti-B-cell therapy (rituximab). Lymphoma-related survival (5-year) depends on subtype and ranges from 97% (ENMZL) to 9% (MCL). Recognizing conjunctival tumors and understanding predisposing factors, biomarkers, and treatment strategies are vital to patient outcomes.

Resección mucosal endoscópica de hiperplasia linfoide reactiva de colon: Presentación de un caso / Endoscopic mucosal resection of reactive lymphoid hyperplasia of colon: One case presentation

La hiperplasia linfoide de colon es una patología gastrointestinal muy poco frecuente. Su tamaño y su forma macroscópica es variable. Se presenta un caso de hiperplasia linfoide reactiva en paciente femenina de 53 años de edad con aspecto macroscópico de una lesión polipoidea sésil tipo IIa ubicada en el ángulo hepático de colon la cual fue tratada con resección mucosal endoscópica. No había evidencia de lesión en otras áreas de la paciente y su evolución fue favorable.

Colon Lymphoid Hyperplasia is a rare gastrointestinal pathology. Its size and its macroscopic form is variable. We present a case of reactive Lymphoid Hyperplasia in a female patient of 53 years old with macroscopic appearance of a sessile polypoid lesion type IIa in the hepatic flexure of colon which was treated with Endoscopic mucosal resection. There was no evidence of the same pathology in other areas of the patient and the evolution of this patient has been favourable.

Enfermedad de Castleman de localización torácica / Intrathoracic Castleman's disease

Antecedentes: la enfermedad de Castleman es conocida como hiperplasia linfática angiofolicular y se caracteriza por crecimiento masivo del tejido linfático. Histológicamente hay tres categorías: hialino-vascular, tipo células plasmáticas y mixto. Se describen dos variantes clínicas: monocéntrica (localizada) y multicéntrica. Objetivo: presentar nuestra experiencia en el manejo de esta enfermedad haciendo énfasis en su compromiso mediastinal y pulmonar. Material y métodos: revisión de historias clínicas e informes patológicos. Resultados: se identificaron 5 pacientes, 1 mujer y 4 hombres, con edad promedio de 58,5 años. Tres pacientes presentaron la forma localizada y dos la multicéntrica. Los síntomas fueron: dolor torácico en dos casos, astenia en 1 caso y síndrome POEMS (polineuropata, organomegalia, endocrinopata y pico monoclo-nal) en 2 casos. Se realizó toracotomía y resección en 2 casos, lobectomía superior derecha en bloque con la lesión mediastinal en 1 caso, biopsia de masa apical en 1 caso y biopsia ganglionar por mediastinoscopia en 1 caso. Se realizó reoperación por sangrado en 1 caso. Tres pacientes presentaron la forma hialino-vascular y 2 la variante de células plasmáticas. Un paciente resecado se perdió de seguimiento. Los pacientes con síndrome POEMS fallecieron, uno al 8° día por neumonía intrahospitalaria y otro al 6° mes por sepsis. Los enfermos resecados restantes cursan buena evolución. Conclusiones: se recomienda la resección quirúrgica en pacientes con enfermedad de Castleman variedad localizada, la cual es curativa. Los pacientes con la variedad multicéntrica no se benefician con esta terapéu-tica, pero son pasibles de quimioterapia o radioterapia o combinación de ambas.

Background: Castleman's disease, known as angio follicular lymph node hyperplasia, is characterized by the massive growth of lymphoid tissue. Histollogically there are three categories: hyaline-vascular, plasm cell type and mixed type. Two clinical variantis have been described: monocentric (localized) and multicentric. Objective: to describe our experience in the treatment of this disease, focusing on itis mediastinal and pulmonary afecton. Method: review of clinical records and pathological reportis. Resultis: 5 patentis (1 female and 4 male), within an age -range of 58, 5 years were screened. Three patentis were afected by the localized type and two by the multicentric type. The symptoms were: thoracic pain in two of the cases, asthenia in one and POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy and monoclonal changes) in 2 cases. We performed thoracotomy and resecton in two cases, upper right side lobectomy in block with the mediastinal lesion in 1 case, apical mass biopsy in 1 case and lymph node biopsy by mediastinoscopy in one case. We performed reoperaton by bleeding in one case. Three patentis presented the hyaline vascular type and two, the plasma-cell type. We lost track of a resectoned patent. Those patentis with POEMS syndrome died, one of them of intra-hospital pneumonia on the eighth day, and another of sepsis on the sixth month. The remaining resectoned patentis are evolving well. Conclusions: on patentis presenting localized type Castleman's disease, we recommend surgical resecton, which is a curative therapy. Patentis presenting the monocentric type do not beneft from this therapy and are passible of chemotherapy and /or radiotherapy.

Approach to Cutaneous Lymphoid Infiltrates: When to Consider Lymphoma?

Cutaneous lymphoid infiltrates (CLIs) are common in routine dermatopathology. However, differentiating a reactive CLI from a malignant lymphocytic infiltrate is often a significant challenge since many inflammatory dermatoses can clinically and/or histopathologically mimic cutaneous lymphomas, coined pseudolymphomas. We conducted a literature review from 1966 to July 1, 2015, at PubMed.gov using the search terms: Cutaneous lymphoma, cutaneous pseudolymphoma, cutaneous lymphoid hyperplasia, simulants/mimics/imitators of cutaneous lymphomas, and cutaneous lymphoid infiltrates. The diagnostic approach to CLIs and the most common differential imitators of lymphoma is discussed herein based on six predominant morphologic and immunophenotypic, histopathologic patterns: (1) Superficial dermal T-cell infiltrates (2) superficial and deep dermal perivascular and/or nodular natural killer/T-cell infiltrates (3) pan-dermal diffuse T-cell infiltrates (4) panniculitic T-cell infiltrates (5) small cell predominant B-cell infiltrates, and (6) large-cell predominant B-cell infiltrates. Since no single histopathological feature is sufficient to discern between a benign and a malignant CLI, the overall balance of clinical, histopathological, immunophenotypic, and molecular features should be considered carefully to establish a diagnosis. Despite advances in ancillary studies such as immunohistochemistry and molecular clonality, these studies often display specificity and sensitivity limitations. Therefore, proper clinicopathological correlation still remains the gold standard for the precise diagnosis of CLIs.

Linfoma ocular de células B da zona marginal: relato de caso e revisão da literatura / Ocular B-cell marginal zone lymphoma: case report and literature review

RESUMO Paciente feminina, 70 anos, apresentou-se com queixas de prurido ocular à esquerda, lacrimejamento e hiperemia há 2 meses. Ao exame físico, apenas hiperemia conjuntival discreta. Foi solicitada biópsia, que sugeriu a hipótese de pseudolinfoma pela insuficiência de critérios classificatórios. Após um ano a paciente retornou com os mesmos sintomas e proptose ocular à esquerda, associado a proliferação de aspecto linfático em conjuntiva bulbar, com presença de vasos sanguíneos, ocupando toda região superior e maior parte da medial, se estendendo até o limbo, sem oclusão do eixo visual e mobilidade ocular extrínseca preservada. Nova biópsia e imuno-histoquímica, foram compatíveis com linfoma de células B da zona marginal. Foram realizadas tomografias computadorizadas (TC) de crânio, pescoço e órbitas, revelando aumento do volume do músculo reto superior esquerdo, com intensa impregnação pelo contraste, acometendo ventre e tendão, com borramento de gordura adjacente. Foram programados 6 ciclos de quimioterapia com ciclofosfamida, doxorrubicina, vincristina e prednisona, CHOP, a cada 21 dias. Houve melhora dos sintomas oculares após o primeiro ciclo. Após o quarto, nova TC de órbitas evidenciou regressão do espessamento do músculo reto superior esquerdo, ainda melhor na TC realizada após o sexto ciclo. Os linfomas MALT são responsáveis por 5-17% de todos os casos de LNH, respondem por mais de 90% dos linfomas que acometem os anexos oculares, mas podem se originar em diversos tecidos. Devido a raridade da doença, carecem estudos prospectivos randomizados que definam um consenso terapêutico. A literatura sugere que o tratamento deve ser individualizado.

ABSTRACT Female patient, 70 years old, presented with complaints of left eye pruritus, tearing and redness, for 2 months. Physical examination revealed only mild conjunctival hyperemia. Biopsy was ordered, and suggested the hypothesis pseudo lymphoma by insufficient classification criteria.After a year, the patient returned with the same symptoms, and left ocular proptosis associated with lymphatic aspect of proliferation in bulbar conjunctiva, with the presence of blood vessels, occupying the entire upper region and most of the medial, extending into the limbo without occlusion the visual axis, extrinsic ocular motility preserved.Another biopsy and immunohistochemistry were compatible with a B cell marginal zone lymphoma. Tomographic studies of cranium, neck and orbits were performed, and the orbit showed enlargement of the left superior rectus muscle volume, with intense contrast uptake, affecting belly and tendon and blurring of adjacent fat. Six cycles of chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisone, CHOP, were done every 21 days. There was improvement in ocular symptoms after the first cycle. After the fourth, new scans from the orbits revealed regression of thickening of the left superior rectus muscle, and even better after the sixth cycle. The MALT lymphomas account for 5-17% of all cases of NHL, accounting for over 90% of lymphomas affecting the eye attachments, but can originate in different tissues. Due to the rarity of the disease, there are no randomized prospective studies to define a therapeutic consensus. The literature suggests that treatment should be individualized.

Alteraciones inmunológicas en la Enfermedad de Castleman / Immunological disorders in Castleman's disease

La enfermedad de Castleman es una entidad poco frecuente, caracterizada por hiperplasia linfoide angiofolicular. Tiene dos tipos histológicos principales: el hialino vascular y el de células plasmáticas; y dos formas clínicas: la localizada y la multicéntrica. Se presenta el caso de una paciente femenina de 19 años de edad con diagnóstico de enfermedad de Castleman tipo hialino vascular, con tres adenopatías localizadas en la región cervical y antecedentes de cuadros infecciosos a repetición previos al diagnóstico. En el estudio inmunohistoquímico se observó CD 20, CD 3 patrón normal y Ki 67 positivo en los folículos linfoides. El estudio inmunológico de sangre periférica arrojó una disminución marcada de los linfocitos CD3+, fundamentalmente a expensas de los CD4+, con conservación de la inmunidad humoral y presencia de anticuerpos antinucleares. Se detectaron anticuerpos IgG anticitomegalovirus y antivirus de Epstein Barr y no se detectaron anticuerpos anti-VIH1,2. Las manifestaciones clínicas sugestivas de inmunodeficiencia antes del diagnóstico de la enfermedad de Casttleman y los resultados de laboratorio obtenidos, permiten sugerir la influencia de la desregulación del sistema inmune en la patogenia de la enfermedad proliferativa. El estado de inmunodeficiencia, además, favorece las infecciones por gérmenes oportunistas, que a su vez potencian los desórdenes inmunes y linfoproliferativos. Se obtuvo una respuesta clínica favorable con tratamiento inmunoestimulante

Castleman'sdisease is a rare disorder characterized by angiofollicularlymphoid hyperplasia. There are two main histological patterns of the disease: the hyaline vascular type and the plasma cell type; andtwo clinical forms: localized and multicentric. We report a case of a 19-year-old female patient with hyaline vascular type Castleman's disease with cervical lymphadenopathies and medical history of repetitive infectious diseases before diagnosis. The inmunohistochemical study reported CD 20, normal pattern of CD3 and Ki 67 positive only in lymphoidfollicles. The immunological study showed low levels of CD3+ CD4+ lymphocytes, slightly low levels of CD3+ CD8+ lymphocytes, and the presence of antinuclear antibodies (ANA). The humoral immunity was not affected.Other findings were IgG antibodies against CMV and EBV positive, and VIH 1,2 antibodies negative. Clinical history suggesting Immunodeficiency and laboratory findings support the hypothesis that deregulation of the immune system could lead to the pathogenesis of the proliferative disease. Also, the immunodeficiency state promotes the onset of opportunistic infections which may increase immune and proliferative disorders. A favorable response to immunostimulant treatment was observed

Doença de Castleman cervical: relato de três casos / Cervical Castleman's disease: report of three cases

A doença de Castleman (DC) é uma rara e benigna desordem linfoproliferativa descrita por Castleman como "hiperplasia hialinizante linfoide". Sua etiologia e patogênese é desconhecida, porém parece haver hiperestimulação antigênica induzida por vírus de forma crônica e mediada por interleucina 6, com alguns casos ligados ao herpes vírus 8 e vírus da imunodeficiência humana (AU)

Castleman's disease (CD) is a rare and benign lymphoproliferative disorder described by Castleman as "hyelinizing lymphoid hyperplasia". Its etiology and pathogenesis are unknown, but there appears to be antigen hyperstimulation chronically induced by virus and mediated by interleukin 6, with some cases related to herpes virus 8 and human immunodeficiency virus (AU)

Enfermedad de Castleman de localización cervical: Caso clínico pediátrico / Cervical Castleman's disease: Pediatric case report

La enfermedad de Castleman es una rara entidad, caracterizada por la hiperplasia de los folículos linfoides. Excepcionalmente afecta a los niños. Presentamos el caso de una niña de 9 años con enfermedad de Castleman en la región parotídea. A pesar de su baja incidencia en la población pediátrica, esta afección puede simular una neoplasia y debe ser tenida en cuenta entre los diagnósticos diferenciales de una masa cervical.

Castleman's disease (CD) is a rare entity, characterized by lymph node follicles hyperplasia. It rarely occurs in children. We present a case of a 9 year old girl with CD in the parotid region. This disease, although it's low incidence in pediatric population, may mimic a malignant neoplasm and should be a differential diagnosis in cervical masses.

Enfermedad de Castleman de localización cervical: Caso clínico pediátrico / Cervical Castlemans disease: Pediatric case report

La enfermedad de Castleman es una rara entidad, caracterizada por la hiperplasia de los folículos linfoides. Excepcionalmente afecta a los niños. Presentamos el caso de una niña de 9 años con enfermedad de Castleman en la región parotídea. A pesar de su baja incidencia en la población pediátrica, esta afección puede simular una neoplasia y debe ser tenida en cuenta entre los diagnósticos diferenciales de una masa cervical.(AU)

Castlemans disease (CD) is a rare entity, characterized by lymph node follicles hyperplasia. It rarely occurs in children. We present a case of a 9 year old girl with CD in the parotid region. This disease, although its low incidence in pediatric population, may mimic a malignant neoplasm and should be a differential diagnosis in cervical masses.(AU)

Hallazgos en ileoscopias realizadas en el servicio de endoscopia del Hospital Universitario de Neiva entre 1996 y 2009 / Findings from ileoscopies performed in the endoscopy service of the Neiva University Hospital from 1996 to 2009

La ileoscopia es la exploración endoscópica del íleon que se puede realizar utilizando un colonoscopio, un enteroscopio de uno o dos balones o una cápsula endoscópica. Tenemos poca información acerca de la patología del íleon en nuestro medio. Objetivo: Describir los hallazgos de ileoscopias practicadas a partir de colonoscopias sin indicación manifiesta y calcular la frecuencia de esos hallazgos. Materiales y métodos: Se revisaron 3.701 informes de colonoscopias practicadas en el Hospital Universitario de Neiva entre 1996 y 2009 y se seleccionaron 417 pacientes para realizar ileoscopia y a 29 de estos se les tomó biopsia. Resultados: Se incluyeron 417 informes de ileoscopias, de los cuales el 92,9% fueron reportados como normales y el 7,1% (34) restante presentó características patológicas. A 28 pacientes de los 34 se les tomó biopsia, y el hallazgo más frecuente fue la inflamación crónica con un 56% e hiperplasia linfoide con 28%. No se informó ninguna complicación por el procedimiento endoscópico. Conclusiones: La ileoscopia es un procedimiento con una baja productividad para patología del íleon distal. No se puede recomendar como una actividad de rutina y su práctica debe dejarse a discreción del endoscopista dependiendo de la disponibilidad de otros métodos, la experiencia y su costo.Ileoscopy is endoscopic exploration of the ileum. It can be performed using two procedures: single or double balloon endoscopy or capsule endoscopy. We have little information about ileum pathologies in our environment.

Objective: Describe findings from ileoscopies performed through colonoscopies without prior indications. Calculate the frequency of these findings. Materials and methods: 3701 reported colonoscopies performed in the Neiva University Hospital were examined between 1996 and 2009. 417 colonoscopies were selected for ileoscopies, which were performed from 29 biopsies. Results: 417 reports were included for analysis. 92.9% were normal. 7.1% (34 patients) presented pathologic characteristics. Of those 34 patients, 28 were chosen for biopsies. Tithe most frequent diagnoses were chronic inflammation (56%) and lymphoid hyperplasia (28%). No complications were reported from the use of endoscopic procedures. Conclusions: Ileoscopy is a procedure with low productivity regarding distal ileum pathologies. We cannot recommend this procedure for routine examinations. It must be practiced with discretion by specialists depending upon the availability of other methods, experience and costs.

Asociación del virus herpes humano 8 y la hiperplasia linfoide nodular difusa del intestino delgado en la inmunodeficiencia variable común / Association between the human herpesvirus 8 and the diffuse nodular lymphoid hyperplasia of the small intestine in common variable immunodeficiency

La inmunodeficiencia variable común (IDVC) es la inmunodeficiencia primaria más frecuente en el terreno clínico y sus formas de presentación son muy variables. Se describe una paciente con IDVC de adulto con síndrome diarreico crónico, pérdida de peso y linfoadenopatías difusas. Sus características inmunológicas más notables fueron una profunda hipogammaglobulinemia de las 3 clases mayores de inmunoglobulinas y la disminución numérica de las células B (CD19+) y células NK (CD3-CD56+) en sangre periférica. La biopsia del intestino delgado obtenida por panendoscopia asistida por video, reveló hiperplasia linfoide multinodular con atrofia parcial de las vellosidades. La inmunohistoquímica mostró que los nódulos consistían en centros germinales aumentados de tamaño con una distribución de células B (CD20+) y células T (CD3+), similar a la del folículo normal. No se encontró expresión diferencial de cadenas ligeras κ y λ. El método de la reacción en cadena de la polimerasa en tiempo real (QRT-PCR) detectó un número apreciable de copias del genoma del virus del herpes humano tipo 8 (VHH-8) (133 copias/µL de ADN) en el ADN del nódulo intestinal biopsiado. La infección con el VHH-8 puede ser un factor importante en la patogenia de los trastornos linfoproliferativos en pacientes con IDVC.

The common variable immunodeficiency (CVID) is the more frequent primary immunodeficiency in clinical field and its presentation forms are very variable. We describe the case of a women presenting with adult CVID with chronic diarrhea syndrome, weight loss and diffuse lymphadenopathies, where the more marked immunologic features were a deep hypogammaglobulinemia of the three major kinds of immunoglobulins and numerical decrease of B cells (CD19+) and NK cells (CD3-CD56+) in peripheral blood. Biopsy of small intestine obtained by video-assisted panendoscope, showed the presence of a multinodular lymphoid hyperplasia with partial atrophy of hairinesses. Immunohistochemistry showed that nodules were high germinal centers with distribution of B cells (CD20+) and T cells (CD3+), similar to that of normal follicle. There was not differential expression of the K and λ light chains. The real time polymerase chain reaction (QRT-PCR) method detected many copies from the genome of type 8 human herpesvirus (VHH-8) (133 copies/µL of DNA) in biopsy of intestinal nodule DNA. VHH-8 infection may to be a significant factor in pathogenesis of lymphoproliferative disorders in patients presenting with CVID.

Utilidad de segunda biopsia de ganglio linfático con informe previo de hiperplasia linfoide reactiva en niños / Usefulness of second lymph node biopsy with a prior report of reactive lymphoid hyperplasia in pediatric patients

Introducción: En niños con ganglios linfáticos superficiales aumentados de tamaño se debe tener cuidado en definir quién requerirá biopsia. El objetivo de esta investigación fue analizar la utilidad de la biopsia de ganglio linfático superficial en el diagnóstico pediátrico. Métodos: De los informes histopatológicos de 364 biopsias de niños y adolescentes se consideró edad, sexo, tiempo de evolución de la adenomegalia, área anatómica, estudios de laboratorio y gabinete, y diagnósticos histopatológicos. Para el análisis estadístico se utilizaron pruebas estadísticas paramétricas y no paramétricas. Resultados: 211 pacientes masculinos (57.9 %) y 153 femeninos (42.0 %); el grupo entre uno y seis años representó 50.8 %, el tiempo promedio de evolución fue de cinco meses, el sitio anatómico predominante fue la región cervical. En 46.9 % se trató de hiperplasia linfoide reactiva seguida de neoplasias (24 %) y procesos granulomatosos (14.5 %); en 14.6 % se incluyeron infecciones diversas; en 95 pacientes con adenopatía en sitios de alarma, 29 eran oncológicos. El coeficiente de determinación de Spearman (R2 = 0.3485) indicó especificidad de patología en esas áreas, aunque la mayoría de los diagnósticos fue de ganglios en regiones diversas; prueba de Fisher con p < 0.0001. En 25 niños con hiperplasia linfoide reactiva en primer estudio, en segunda biopsia se estableció diagnóstico; la probabilidad binomial fue p = 0.0493, con 75 % de certeza y prueba de χ2 con p = 0.0512. Conclusiones: Los ganglios en regiones de alarma indican probabilidad para encontrar enfermedad; en crecimiento persistente con informe inicial de hiperplasia linfoide reactiva debe considerarse segunda biopsia pues existe probabilidad > 75 % de establecer la causa.

BACKGROUND: Physicians in daily practice frequently attend children who present an increase in lymph node size. Care should be taken to determine which children require biopsy. Our objective was to analyze the usefulness of the peripheral lymph node biopsy in pediatric diagnosis. METHODS: We reviewed histological reports of 364 lymph node biopsies. Variables were age, sex, lymphadenopathy evolution time, laboratory and imaging studies, and histopathology diagnoses. Parametric and nonparametric tests were used. RESULTS: There were 211 males (57.9 %) and 153 females (42.0 %), the 1- to 6-year age group included 50.8% of cases. Average disease-evolution time was 5 months, and the predominant anatomic site was the cervical region. Histological report was reactive lymphoid hyperplasia (RLH) in 46.9%, followed by neoplasms in (24%), and granulomas (14.5%). Other diagnoses and normal lymph nodes represented 14.6 %. Of the 95 patients with high-risk lymphadenopathy, 29 cases had oncological diagnoses. Spearman coefficient was R(2) = 0.3485. To obtain a specific diagnosis indicates the probability of disease in lymph nodes of risk areas. However, the majority of the diagnoses were obtained from lymph nodes in different areas (Fisher's test, p <0.001). In 25 children with RLH, the binominal probability of diagnosis in the second biopsy was p = 0.0493. There was a >75% chance for detecting disease. CONCLUSIONS: Peripheral lymph nodes in high-risk regions indicate probability relationship for determining disease. In persistent lymphadenopathy with an initial report of RLH, it is important to consider a second biopsy with high probability for obtaining a specific diagnosis.