Histiocitosis de células de Langerhans congénita


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Title Histiocitosis de células de Langerhans congénita
Histiocitosis de células de Langerhans congénita: reporte de caso
Creator Barrios , Katherine
Patiño, Oscar
Muñoz, Nelson
Moneriz, Carlos
Subject Langerhans Cell, Histiocytosis, Newborn, Infant, Congenital, Colombia
Célula de Langerhans; histiocitosis; recién nacido; lactante; congénita, Colombia
Description Langerhans cell histiocytosis (LCH) is a rare pathology, with different clinical manifestations in the neonatal period, ranging from isolated bone lesions to a systemic compromise. A case of Langerhans cell histiocytosis is reported with literature review focusing on clinical manifestations, diagnosis and treatment. One month old patient consulted for presenting lymphadenopathy and skin lesions, which was initially managed as an infectious pathology. The disease continued its progression and without improvement with the treatment the patient dies due to respiratory failure. The lymph node and skin biopsy revealed infiltration of atypical cells with immunohistochemistry positive for S100, CD1 and CD68, confirming the LCH. This alteration is a rare disease of low incidence with a variety of clinical manifestations, representing a great challenge. Therefore, it is important to alert and sensitize the medical team about this disease for a diagnosis and timely management.
La histiocitosis de células de Langerhans (LCH) es una patología poco frecuente, con diferentes manifestaciones clínicas que pueden iniciar en el periodo neonatal, estas varían desde lesiones óseas aisladas hasta un compromiso sistémico. Se describe un caso de histiocitosis de células de Langerhans y se revisa la literatura centrada en manifestaciones clínicas, diagnóstico y tratamiento. El paciente de un mes de nacido consultó por presentar adenopatías y lesiones en piel, el cual fue manejado inicialmente como patología infecciosa. La enfermedad continuó su progresión y sin mejoría con el tratamiento, hasta su fallecimiento por falla respiratoria. La biopsia de ganglio y piel reveló infiltración de células atípicas y la inmunohistoquímica resultó positiva para proteína S100, CD1 y CD68, confirmándose con esto el diagnostico de LCH. Esta alteración es una enfermedad rara de baja incidencia con un gran abanico de manifestaciones clínicas, representando un gran desafío. Por lo tanto, es importante alertar y sensibilizar al equipo médico sobre esta enfermedad para un diagnóstico y manejo más oportuno.
Publisher Instituto Nacional de Salud
Date 2020-04-17
Type info:eu-repo/semantics/article
Format application/pdf
Identifier https://revistabiomedica.org/index.php/biomedica/article/view/5150
Source Biomedica; Vol. 40 No. 3 (2020): Septiembre, publicación anticipada
Biomédica; Vol. 40 Núm. 3 (2020): Septiembre, publicación anticipada
Language spa
Relation https://revistabiomedica.org/index.php/biomedica/article/view/5150/4555
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Rights Derechos de autor 2020 Biomédica

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