Resumen
Objetivo: Reportar un caso clínico de un paciente joven diagnosticado con Enfermedad de Rasai-Dorfman, una patología rara, con afectación ganglionar inusual. Detalles del caso: Paciente masculino de 15 años de edad, ingresó a urgencias vía urgencias por cuadro de astenia, disnea de medianos y ligeros esfuerzos asociada a pérdida de peso y dolor torácico dependiente de ventilación de aproximadamente 30 días de evolución, que evolucionó a intensificación. de síntomas, cianosis de extremidades y esputo hemoptoico. Tras la investigación clínica, el estudio inmunohistoquímico reveló un patrón de enfermedad de Rasai-Dorfman. Luego, el paciente pasa al seguimiento ambulatorio y a la decisión clínica sobre el tratamiento. Consideraciones finales: También conocida como histiocitosis sinusal con linfadenopatía masiva (SHML), la enfermedad de Rasai-Dorfman es poco común y el plan terapéutico aún es controvertido. En el caso reportado, la cadena linfática en el mediastino resultó afectada, sin afectar los ganglios linfáticos cervicales, algo inusual en esta rara enfermedad. Se destaca la importancia del estudio multidisciplinar de esta patología con el objetivo de proporcionar un diagnóstico precoz en otros casos e incentivar la investigación del tratamiento ofrecido.
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