Histiocitosis de células de Langerhans en niños. Descripción de 10 casos

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Abstract

Introducción: la histiocitosis de células de Langerhans constituye una entidad poco frecuente, con diferentes tipos de presentación clínica y patológica, que varían desde unisistémica y unifocal hasta compromiso de múltiples sistemas. Es consecuencia de la proliferación, acúmulo e infiltración en diferentes órganos, de células que hacen parte del sistema fagocítico mononuclear. El objetivo con la descripción de esta serie de casos es la de resaltar la importancia y sensibilizar a un diagnóstico y manejo oportuno de esta entidad, para una mejor calidad de vida y mayor sobrevida. Métodos: se realizó un estudio retrospectivo en pacientes pediátricos diagnosticados en la Fundación Valle del Lili en un periodo de seis años. Resultados: se registraron 10 casos de la enfermedad. No se observaron diferencias por sexo. La edad media de presentación fue de 33 meses. En el diagnóstico por inmuno-histoquímica se identificó histiocitosis de células de Langerhans con compromiso multisistemico -multifocal en cinco casos, uno con compromiso unisistémico - unifocal y otro unisistémica - multifocal y granuloma eosinofílico en los tres restantes. Cinco pacientes presentaron compromiso de órgano de riesgo y uno de ellos murió en el periodo de observación. Conclusión: la histiocitosis de células de Langerhans es una enfermedad de compromiso sistémico muy poco frecuente en pediatría, con diversas formas de presentación clínica, lo que la hace una entidad de difícil diagnóstico y manejo. La inmunohistoquímica es la mejor forma de diagnóstico en esta entidad

Translated abstract

Introduction: Langerhans cell histiocytosis is a rare disease with different clinical and pathological presentation, is clinically divided into three groups: unifocal, multifocal unisystem, and multifocal multisystem Langerhans cell histiocytosis is a consequence of proliferation, accumulation and infiltration of cells that are part of the mononuclear phagocyte system in different organs. The goal with the description of this cases series is to highlight the importance and awareness to a timely diagnosis and management of this entity, for a better quality of life and longer survival. Methods: Retrospective study in pediatric patients diagnosed in the Valle del Lili Foundation over a period of six years. Results: In our series of cases there was no gender difference and the mean age at presentation was 33 months. In the diagnosis by immunohistochemistry identified Langerhans Cell Histiocytosis, the cases were classified as multisystemic -multifocal in 5 patients, unisystemic in 1 patients - unifocal unisystemic in 1 patients -multifocal eosinophilic granuloma in 3 patients. Of the 10 patients 5 had risk organ involvement and 1 died in the observation period. Discussion: la histiocitosis de células de Langerhans es una enfermedad de compromiso sistémico muy poco frecuente en pediatría, de diversas formas de presentación clínica, lo que la hace una entidad de difícil diagnóstico y manejo. La inmunohistoquimica es la mejor forma de diagnóstico en esta entidad

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Central diabetes insipidus as the inaugural manifestation of Langerhans cell histiocytosis: natural history and medical evaluation of 26 children and adolescents.

I Marchand, M Barkaoui, Catherine Garel … (2011)

Isolated central diabetes insipidus (CDI) can be the first manifestation of Langerhans cell histiocytosis (LCH), creating diagnostic dilemmas such as dysgerminoma and other inflammatory lesions. In 2010, the French national LCH registry had enrolled 1236 LCH patients under 18 yr of age. Isolated CDI was the initial presentation of LCH in 26 patients. We reviewed their clinical and magnetic resonance imaging (MRI) features. Median age at the diagnosis of CDI was 9.6 yr (1.8-16.3), and median follow-up after CDI diagnosis was 9.9 yr (3.5-26.6). In addition to CDI, two patients had visual field defects, four had secondary amenorrhea, and 11 had anterior pituitary deficiency. Cerebral imaging (including computed tomography in two cases), performed in 22 patients within 3 months of CDI diagnosis, showed pituitary stalk thickening in 14 patients, which was moderate (3.0-7 mm) in nine cases and marked (>7 mm) in five cases. In eight cases, the lesion extended to the floor of the third ventricle. One child with LCH presented with a mild enlarged sellar content. During follow-up, 22 patients developed extrapituitary involvement, mainly of bone (n = 15), lung (n = 9), and skin (n = 9). Pituitary biopsy was performed in eight cases and was conclusive in six cases. Pituitary stalk thickening can be observed in LCH as well as lesions extending to the floor of the third ventricle. In all cases but one, the intrasellar content was not enlarged. Long-term follow-up with close attention to bone, skin, and lung disorders may lead to the diagnosis of LCH.