ABSTRACT
ABSTRACT: Granulomatous slack skin (GSS) is a rare subtype of mycosis fungoides, and few cases have been known to spread to the blood, lymph nodes, or viscera. We present a case with early dissemination to the lung. A 27-year-old woman, previously healthy, presented with scattered disseminated scaly patches, associated with vulvar and intergluteal firm swelling and groin-skin induration for 1 year. She also reported mild fatigue and breathlessness on moderate exertion. The patient underwent blood tests, skin biopsies, and computed tomography scan. The skin biopsy showed a mildly atypical T-cell lymphoid infiltrate involving the dermis/hypodermis, with focal epidermotropism, associated with a granulomatous infiltrate and elastophagocytosis. The computed tomography scan revealed bilateral ground-glass lung nodular opacities. Positron emission tomography showed an increased signal in the skin and subcutis around the buttocks, inguinal and mediastinal lymph nodes, and lungs. The lung biopsy confirmed a dense T-cell infiltrate with numerous multinucleated giant cells. Subsequently, esophageal involvement was also observed following biopsy. Molecular analyses demonstrated identical T-cell clones in the skin and lung. After 6 cycles of chemotherapy/localized external radiotherapy, the patient had a partial skin response and stable lung disease. A preferred diagnosis of GSS with systemic spread was made based on clinical/histologic/molecular findings, after considering granulomatous mycosis fungoides and peripheral T-cell lymphoma, not otherwise specified. This case highlights the frequent diagnostic difficulty in distinguishing GSS from an inflammatory granulomatous dermatitis. Pulmonary and esophageal involvements are rare in GSS, and the simultaneous presentation of characteristic cutaneous GSS with systemic disease poses an additional classification challenge.
ABSTRACT
La Enfermedad Granulomatosa Crónica es una inmunodeficiencia hereditaria en la cual la capacidad microbicida de las células fagocíticas está alterada debido a defectos en la enzima Nicotinamida dinucleótido fosfato oxidasa. El diagnóstico de esta enfermedad puede realizarse por citometría de flujo, demostrando una deficiente producción de intermediarios reactivos del oxígeno en los fagocitos mediante la prueba de Dihidrorhodamina 123. Objetivo: El propósito de este trabajo es determinar valores de referencia para la técnica la prueba de Dihidrorhodamina 123 por citometría de flujo en el Hospital de Niños. Métodos: Se evaluó el estallido respiratorio de neutrófilos en muestras pertenecientes a niños saludables (n=48), mediante las técnicas de Nitroblue de Tetrazolium y de Dihidrorhodamina 123. Resultados: Todas las muestras presentaron valores normales de Nitroblue de Tetra;wlium. Los resultados de la técnica de Dihidrorhodamina 123 se expresaron en términos de Indice de Estimulación, cuyo promedio y desviación estándar fueron 27.6 ± 10.2, Y el intervalo de referencia de 10.1 a 57.8. Conclusiones: El intervalo de referencia para la técnica de Dihidrorhodamina 123 determinado en el Laboratorio del Hospital de Niños difiere de otros hallados en la bibliografía, ya que estos valores se ven afectados por condiciones técnicas propias y el tipo de citómetro utilizado.
Subject(s)
Flow Cytometry , Granulomatous Disease, Chronic , Respiratory BurstABSTRACT
O experimento foi conduzido com o objetivo de avaliar o efeito da adição de um compelxo multienzimático exógeno composto de amilase, protease, lipase e celulase, em rações de juvenis de tambaqui, sobre os coeficientes de digestibilidade aparente (CDa) da proteína bruta (PB), extrato etéreo (EE), carboidratos (ENN) e energia bruta (EB). O delineamento experimental foi inteiramente casualizado com quatro tratamentos (quatro níveis de inclusão de enzimas, 0,0; 0,05; 0,10; e 0,15 por cento), três repetições (no tempo) e 10 peixes por unidade experimental. Foram utilizados 40 juvenis de tambaqui, com peso médio de 155,0 ± 0,49 g, distribuídos em quatro tanques de alimentação de 500 l, recebendo refeições à vontade das 8 às 12h, a cada hora. Em seguida os animais foram transferidos para coletores de fezes (200 l), onde permaneceram até às 18h, sendo a coleta de dejetos realizada a cada hora. A determinação dos CDa foi realizada pelo método indireto, sendo utilizado como indicador externo 0,5 por cento de óxido de cromo-III (Cr2O3) incorporado à ração. Os resultados demonstraram que a suplementação das dietas com enzimas exógenas para juvenis de tambaqui aumenta a digestibilidade aparente dos nutrientes e energia bruta, no nível de inclusão de 0,05 por cento (P<0,05 por cento).
The experiment evaluated the effect of dietary supplementation a multienzyme complex containing amylase, protease, lipase and cellulase for tambaqui juveniles on the apparent digestibility coefficients (ADCs) of crude protein (CP), ether extract (EE), carbohydrates (CHO) and crude energy (CE). A completely randomized experimental design was used with four treatments (level of enzymes 0.0; 0.05; 0.10; and 0.15 percent) and three replicates (over time), with ten fish per experimental unity. Forty fish weighting 155.0 ± 0.49g were distributed in four 500 l feeding tanks and were fed hourly, from 8 to 12 AM. After this period, fish were transferred for fecal collection units until 6 PM, where feces were collected hourly. ADCs were evaluated by an indirect method, with 0.5 percent III-chromic oxide (Cr2O3) as innert marker. The results showed that supplementary exogenous enzymes on tambaqui diets increased the apparent digestibility of nutrients and crude energy at inclusion level of 0.05 percent (P<0.05 percent).
