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Medicina & Laboratorio

2021, Número 4

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Medicina & Laboratorio 2021; 25 (4)


Histiocitosis de células de Langerhans, una afección pulmonar infradiagnosticada en fumadores: reporte de caso y revisión de la literatura

Cabrera-Rojas DL, Gómez-Correa GA, Varela-Aguirre GJ

Idioma: Español
Referencias bibliográficas: 60
Paginas: 721-734
Archivo PDF: 299.60 Kb.


RESUMEN

La histiocitosis de células de Langerhans (HCL) es una enfermedad que puede afectar a pacientes de cualquier edad, siendo en adultos un trastorno poco común de etiología desconocida, que ocurre predominantemente en fumadores jóvenes, sin diferencias en género. Aunque ciertas particularidades de la enfermedad pueden compartirse con las manifestaciones presentes en la población pediátrica, la proporción de casos con afectación pulmonar es mucho mayor en adultos. A menudo evoluciona a través de brotes sucesivos y su gravedad varía desde formas benignas hasta potencialmente mortales. Algunos pacientes desarrollan un importante deterioro funcional con repercusión psicosocial, que impacta en la calidad de vida y se asocia a discapacidad prolongada. La clave diagnóstica estará determinada por el antecedente de tabaquismo, la presencia de nódulos, nódulos cavitados y quistes de paredes gruesas y delgadas en la tomografía computarizada de tórax de alta resolución (TACAR). Sin embargo, el diagnóstico definitivo requiere la identificación de granulomas de células de Langerhans, que generalmente se logra mediante la realización de una biopsia pulmonar y su estudio histopatológico e inmunohistoquímico. En la actualidad, podríamos considerar a esta entidad como una enfermedad huérfana, de la cual aún no se tiene claridad del mecanismo patogénico, y que, por ende, aún no dispone de estrategias terapéuticas específicas. El objetivo de esta revisión está centrado en la aproximación diagnóstica y terapéutica de la histiocitosis de células de Langerhans en adultos, que permita facilitar su reconocimiento en etapas tempranas y mejorar el pronóstico en las personas que la padecen.


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