Histiocitosis de células de Langerhans congénita

Biomédica

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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
info:eu-repo/semantics/publishedVersion
 
Format application/pdf
 
Identifier https://revistabiomedica.org/index.php/biomedica/article/view/5150
10.7705/biomedica.5150
 
Source Biomedica; Vol. 40 No. 3 (2020): Septiembre, publicación anticipada
Biomédica; Vol. 40 Núm. 3 (2020): Septiembre, publicación anticipada
2590-7379
0120-4157
 
Language spa
 
Relation https://revistabiomedica.org/index.php/biomedica/article/view/5150/4555
/*ref*/Morimoto A, Oh Y, Shioda Y, Kudo K, Imamura T. Recent advances in Langerhans cell histiocytosis. Pediatrics international : official journal of the Japan Pediatric Society. 2014;56(4):451-61. 2. Filipovich A, McClain K, Grom A. Histiocytic disorders: recent insights into pathophysiology and practical guidelines. Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation. 2010;16(1 Suppl):S82-9. 3. Jaffe R. The diagnostic histopathology of Langerhans cell histiocytosis. In: Egeler RM, Weitzman S, editors. Histiocytic Disorders of Children and Adults: Basic Science, Clinical Features and Therapy. Cambridge: Cambridge University Press; 2005. p. 14-39. 4. Jezierska M, Stefanowicz J, Romanowicz G, Kosiak W, Lange M. Langerhans cell histiocytosis in children - a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment. Postepy dermatologii i alergologii. 2018;35(1):6-17. 5. Abla O, Egeler RM, Weitzman S. Langerhans cell histiocytosis: Current concepts and treatments. Cancer Treatment Reviews. 2010;36(4):354-9. 6. Grana N. Langerhans cell histiocytosis. Cancer control : journal of the Moffitt Cancer Center. 2014;21(4):328-34. 7. Salotti JA, Nanduri V, Pearce MS, Parker L, Lynn R, Windebank KP. Incidence and clinical features of Langerhans cell histiocytosis in the UK and Ireland. Archives of Disease in Childhood. 2009;94(5):376-80. 8. Castro ECC, Blazquez C, Boyd J, Correa H, de Chadarevian J-P, Felgar RE, et al. Clinicopathologic Features of Histiocytic Lesions following ALL, with a Review of the Literature. Pediatric and Developmental Pathology. 2010;13(3):225-37. 9. Krooks J, Minkov M, Weatherall AG. Langerhans cell histiocytosis in children: Diagnosis, differential diagnosis, treatment, sequelae, and standardized follow-up. J Am Acad Dermatol. 2018;78(6):1047-56. 10. Frade AP, Godinho MM, Batalha ABW, Bueno APS. Congenital Langerhans cell histiocytosis: a good prognosis disease? Anais Brasileiros de Dermatologia. 2017;92:40-2. 11. Grois N, Pötschger U, Prosch H, Minkov M, Arico M, Braier J, et al. Risk factors for diabetes insipidus in langerhans cell histiocytosis. Pediatric Blood & Cancer. 2006;46(2):228-33. 12. Merglova V, Hrusak D, Boudova L, Mukensnabl P, Valentova E, Hosticka L. Langerhans cell histiocytosis in childhood - review, symptoms in the oral cavity, differential diagnosis and report of two cases. Journal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial Surgery. 2014;42(2):93-100. 13. Krooks J, Minkov M, Weatherall AG. Langerhans cell histiocytosis in children: History, classification, pathobiology, clinical manifestations, and prognosis. J Am Acad Dermatol. 2018;78(6):1035-44. 14. Kapur P, Erickson C, Rakheja D, Carder KR, Hoang MP. Congenital self-healing reticulohistiocytosis (Hashimoto-Pritzker disease): Ten-year experience at Dallas Children's Medical Center. Journal of the American Academy of Dermatology. 2007;56(2):290-4. 15. Berres ML, Merad M, Allen CE. Progress in understanding the pathogenesis of Langerhans cell histiocytosis: back to Histiocytosis X? British journal of haematology. 2015;169(1):3-13. 16. Hutter C, Minkov M. Insights into the pathogenesis of Langerhans cell histiocytosis: the development of targeted therapies. ImmunoTargets and therapy. 2016;5:81-91.
 
Rights Derechos de autor 2020 Biomédica
https://creativecommons.org/licenses/by/4.0
 

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