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.

A Descriptive Study to Assess the Prevalence of Appendicitis in a Known Population

Background: Acute appendicitis is the most common surgicaldisease, and appendectomy is the treatment of choice in themajority of cases. The present study was conducted to assessthe prevalence of Appendicitis in a known population.Materials and Methods: The present study was conductedamong 140 patients of age group 20-60 years. The patient whowere diagnosed as Appendicitis by symptoms and confirmedby ultrasonography over the period of 6 months. Thesepatients were operated and confirmed by histopathologically.All the necessary information was collected by pretested, semistructured questionnaire.Results: In the present study proportion of male patients(53.57%) was more as compared to females (46.4%). Themost common age for appendicitis was found to be 20-30 i.e.(45%) followed by 31-40 (25%).41-50 (20%), 51-60 (10%). Themost common presenting complain was Pain in Abdomen(100%) followed by Fever (75.71%), Vomiting (52.14%). Inhistopathological diagnosis the most common diagnosis wasAcute Appendicitis (60%) followed by lymphoid hyperplasia(30%).Conclusion: Our study concluded that males were affectedmore by appendicitis, most common age for appendicitis was20-30 years, the most presenting complain was pain inabdomen and histological diagnosis shows that acuteappendicitis was most common.

Chronic cholecystitis with follicular lymphoid hyperplasia: nomenclature and diagnostic dilemmas

Background: To revisit the nomenclature, prevalence, histogenesis and the diagnostic dilemmas in cases of cholecystitis with lymphoid hyperplasia received in a private laboratory in one-year duration.Methods: A total of 51 cases of cholecystectomy were examined histopathologically to identify and review all the cases with emphasis on cholecystitis with marked lymphoid infiltration.Results: Out of 51 cholecystectomy specimens, some rare entities were observed such as 4 cases (8%) of xanthomatous change, 2 cases (4%) of cholecystitis with follicular lymphoid hyperplasia and a case of hyalinizing cholecystitis.Conclusions: The literature on cholecystitis with marked lymphoid infiltrate (with or without follicle formation) was overlapping and thus confusing. The same has been simplified with review of literature.

Mucosa-associated lymphoid tissue lymphoma on right lower eyelid previously diagnosed as lymphoid hyperplasia

Mucosa-associated lymphoid tissue (MALT) lymphoma is an uncommon form of non-Hodgkin lymphoma involving the mucosa-associated lymphoid tissue. Most commonly affected organ is the stomach. But, it could affect almost every organ in the whole body. If they have suspicious lesion, excision biopsy could be made. For staging, blood test including blood smear, abdomen and chest tomography or scan will be checked. Also, bone marrow test can be done if it is needed. The patient had visited the clinic for palpable mass on right lower eyelid. With excisional biopsy, it was diagnosed as lymphoid hyperplasia on pathologic test. But 2 years later, the patient came with recurrent symptom for our department with worry. At that time, we recommended excisional soft tissue biopsy under general anesthesia. Unfortunately, it was revealed MALT lymphoma on pathologic finding. It turned out to be stage 3 in Ann Arbor staging system without B symptoms. Hematologic consultation was made and she was treated with adjuvant chemotherapy for eight cycles to complete remission. We report a case of MALT lymphoma on subcutaneous tissue at right lower eyelid previously diagnosed as lymphoid hyperplasia.

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)

Pulmonary Nodular Lymphoid Hyperplasia with Mass-Formation: Clinicopathologic Characteristics of Nine Cases and Review of the Literature

BACKGROUND: Pulmonary nodular lymphoid hyperplasia (PNLH) is a non-neoplastic pulmonary lymphoid disorder that can be mistaken for malignancy on radiography. Herein, we present nine cases of PNLH, emphasizing clinicoradiological findings and histological features. METHODS: We analyzed radiological and clinicopathological features from the electronic medical records of nine patients (eight females and one male) diagnosed with PNLH. IgG and IgG4 immunohistochemical staining was performed in three patients. RESULTS: Two of the nine patients had experienced tuberculosis 40 and 30 years prior, respectively. Interestingly, none were current smokers, although two were ex-smokers. Three patients complaining of persistent cough underwent computed tomography of the chest. PNLH was incidentally discovered in five patients during examination for other reasons. The remaining patient was diagnosed with the disease following treatment for pneumonia. Imaging studies revealed consolidation or a mass-like lesion in eight patients. First impressions included invasive adenocarcinoma and mucosal-associated lymphoid tissue‒type lymphoma. Aspergillosis was suspected in the remaining patient based on radiological images. Resection was performed in all patients. Microscopically, the lesions consisted of nodular proliferation of reactive germinal centers accompanied by infiltration of neutrophils and macrophages in various degrees and surrounding fibrosis. Ultimately, all nine patients were diagnosed with PNLH and showed no evidence of recurrence on follow-up. CONCLUSIONS: PNLH is an uncommon but distinct entity with a benign nature, and understanding the radiological and clinicopathological characteristics of PNLH is important.

Twin Rectal Tonsils Mimicking Carcinoid or Mucosa-Associated Lymphoid Tissue Lymphoma

The rectal tonsil is a rare polypoid lesion exclusively found in the rectum and is considered a reactive proliferation of the lymphoid tissue. Although this lesion is benign, we recommend that it should be differentiated from carcinoid or polypoid type of mucosa-associated lymphoid tissue lymphomas, based on gross findings. In this case report, we describe a case of rectal lesions with a unique appearance in a 41-year-old man. Colonoscopy revealed two 5-mm-sized nodules located opposite from each other on the left and right sides of the lower rectum. Endoscopic mucosal resection was conducted. Histopathologically, both lesions were mainly located in the submucosa and consisted of prominent lymphoid follicles with germinal centers of various sizes. No immunoreactivity of Bcl-2 was seen in the germinal centers. Immunohistochemical staining for kappa and lambda light chains revealed a polyclonal pattern. Therefore, these lesions were diagnosed as rectal tonsils.

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.

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.

Lymphocytoma cutis: diagnostic enigma for the maxillofacial surgeon

Cutaneous lymphoid hyperplasia (CLH) is a cutaneous pseudolymphoma with a worldwide distribution, equally affecting all races and ethnic groups. Due to its vast array of characteristics, it is most often missed in the differential diagnosis of firm to soft lumps on the head and neck. A systematic approach to the workup and diagnosis along with treatment of such lesions is discussed in this article. A 20-year-old Asian Indian female presented to our Oral and Maxillofacial unit with a lump on the left side of her forehead for 1 month. Local examination revealed a 2.5×3.0 cm², well circumscribed swelling over the left para median region that was firm to doughy and non-tender. There was no other significant finding on general examination. Excisional biopsy of the lesion was performed, followed by histopathologic processing. The general etiology, pathogenesis, clinical presentation, differential diagnosis, clinical course, prognosis, treatment, and prevention have been discussed in line with the recent modalities of diagnosis and treatment of CLH. Due to the overlapping clinical and histological characteristics of CLH with many other lesions, it is important to consider this lesion in the differential diagnosis of cutaneous lesions.

Clinicopathologic study of primary thymic extranodal marginal zone lymphoma of mucosa associated lymphoid tissue and lymphoepithelial sialadenitis-like thymic hyperplasia / 临床与实验病理学杂志

Purpose To discuss the clinicopathological features of primary thymic extranodal marginal zone lymphoma of mucosa associated lymphoid tissue (MALT) lymphoma and lymphoepithelial sialadenitis (LESA)-like thymic hyperplasia,their relationship and differential diagnosis.Methods Three cases of thymic MALT lymphoma and one LESA-like thymic hyperplasia were evaluated by HE staining,immunohistochemistry and immunoglobulin (Ig) gene rearrangement technology.Results The symptoms of Sjsgren syndrome were found in the two patients with thymic MALT lymphoma.Microscopically,the normal architecture of thymus was effaced by dense lymphoid infiltration composed predominantly of centrocyte-like and monocytoid B cells with prominent lymphoepithelial lesions.Immunohistochemically,the tumor cells were positive CD20,PAX-5,and BCL-2.The plasma cells showed lambda light chain restriction in one case with prominent plasmacytoid differentiation.In LESA-like thymic hyperplasia,the normal lobular architecture of thymus was generally reserved and abundant lymphoid tissue containing lymphoid follicles was seen with prominent lymphoepithelial lesions in expanding islands of thymic epithelial cells and epithelium lining the cysts,but a monocytoid B-cell population was absent.Immunohistochemically,a mixed B-cell and T-cell population was identified.A monoclonal rearrangement of the Ig gene was detected in all three thymic MALT lymphomas but not in the case of LESA-like hyperplasia.Conclusion Primary thymic MALT lymphoma and LESA-like thymic hyperplasia are both rare lymphoid proliferative lesions and the two lesions have overlapping histological and immunohistochemical features.A combination of genetic rearrangement and analysis of the differential points is helpful to distinguish between them.

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)

Clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric Castleman's disease: a single medical center experience

BACKGROUND: Multicentric Castleman's disease (CD) is commonly associated with poor prognosis, and well-known prognostic factors are scarce. We performed a retrospective analysis to define the clinical features and prognostic factors for patients with multicentric CD. METHODS: Between 1990 and 2013, 32 patients with multicentric CD were identified from the database of the Asan Medical Center, Seoul, Korea. Clinicopathologic data were collected by reviewing the medical records. With the exclusion of 4 patients because of unknown human immunodeficiency virus infection status, 28 human immunodeficiency virus-negative patients with multicentric CD were included in this analysis. RESULTS: Most of the patients were male (76%) and had a median age of 54 years. Hyaline vascular variant was the most common subtype (N=11, 39%). Hepatosplenomegaly (61%), fever (39%), edema (29%), and ascites (18%) were the most frequently reported symptoms and signs at diagnosis. With a median follow-up of 67 months, the 5-year overall survival (OS) was 77%. Patients with extravascular fluid accumulation (i.e., peripheral edema, ascites, and/or pleural effusions) were significantly associated with a poor survival rate (5-year OS, 94% vs. 56%; P=0.04). The extent of disease involvement was also a significant prognostic factor (5-year OS, 91% for involvement on a single side vs. 73% on both sides of the diaphragm; P=0.03). Other clinicopathologic factors were not significantly associated with patient survival. CONCLUSION: Our findings suggest that the hyaline vascular variant is not a rare subtype of multicentric CD. Extravascular fluid accumulation and disseminated disease involvement seem to be significant prognostic factors.

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.

Reactive lymphoid hyperplasia of the liver

No abstract available.