Late Diagnosis of Langerhans Cell Histiocytosis by Skin Biopsy in a Lung Transplant Candidate Patient

Autores
Klein, Francisco R.; Klein, Julia; Otalora Lozano, Diego; Vigliano, Carlos
Año de publicación
2024
Idioma
inglés
Tipo de recurso
artículo
Estado
versión publicada
Descripción
Langerhans cell histiocytosis (LCH) is a rare disease. In the adult population, isolated pulmonary presentation predominates. The course of the disease can be variable, ranging from mild and self-limited cases to potentially fatal respiratory manifestations, with some individuals requiring lung transplantation. A 20-year-old patient started 3 years before his admission with progressive dyspnea leading to a diagnosis of bullous emphysema of undetermined cause, which evolved to respiratory failure and evaluation for bilateral pulmonary transplantation. Three years later, he developed bilateral pneumonia requiring mechanical ventilation. When refractory hypoxemia ensued, he had to be placed on extracorporeal veno-venous membrane oxygenation assistance (VV ECMO). Under these conditions he was transferred to our center and included in the waiting list for a bilateral pulmonary transplantation. Forty-eight hours after admission, and due to intense polyuria, central diabetes insipidus was diagnosed. In this clinical context, the presence of cutaneous lesions on the scalp were reconsidered and biopsied under the presumption of possible LCH, with pathology analysis confirming the diagnosis. He continued to be assisted with VV ECMO for 66 more days as a bridge to transplantation, developing multi-organ failure and passing away before a donor organ was available. The diagnosis of LCH should be considered in any adult patient with bullous emphysema of undetermined cause. Given the possibility of early therapeutic interventions, the search for its clinical associations (such as diabetes insipidus and/or skin lesions) should be a systematic part of the etiologic workup. The availability of skin specimens to reach a diagnosis make its thorough search an important part of the diagnostic approach.
Fil: Klein, Francisco R.. Fundación Favaloro; Argentina
Fil: Klein, Julia. Sheba Medical Center; Israel
Fil: Otalora Lozano, Diego. Fundación Favaloro; Argentina
Fil: Vigliano, Carlos. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Medicina Traslacional, Trasplante y Bioingeniería. Fundación Favaloro. Instituto de Medicina Traslacional, Trasplante y Bioingeniería; Argentina
Materia
LANGERHANS CELL HISTIOCYTOSIS
SKIN BIOPSY
LUNG TRANSPLANTATION
ACUTE RESPIRATORY FAILURE
Nivel de accesibilidad
acceso abierto
Condiciones de uso
https://creativecommons.org/licenses/by/2.5/ar/
Repositorio
CONICET Digital (CONICET)
Institución
Consejo Nacional de Investigaciones Científicas y Técnicas
OAI Identificador
oai:ri.conicet.gov.ar:11336/256986

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network_name_str CONICET Digital (CONICET)
spelling Late Diagnosis of Langerhans Cell Histiocytosis by Skin Biopsy in a Lung Transplant Candidate PatientKlein, Francisco R.Klein, JuliaOtalora Lozano, DiegoVigliano, CarlosLANGERHANS CELL HISTIOCYTOSISSKIN BIOPSYLUNG TRANSPLANTATIONACUTE RESPIRATORY FAILUREhttps://purl.org/becyt/ford/3.2https://purl.org/becyt/ford/3https://purl.org/becyt/ford/3.2https://purl.org/becyt/ford/3https://purl.org/becyt/ford/3.1https://purl.org/becyt/ford/3Langerhans cell histiocytosis (LCH) is a rare disease. In the adult population, isolated pulmonary presentation predominates. The course of the disease can be variable, ranging from mild and self-limited cases to potentially fatal respiratory manifestations, with some individuals requiring lung transplantation. A 20-year-old patient started 3 years before his admission with progressive dyspnea leading to a diagnosis of bullous emphysema of undetermined cause, which evolved to respiratory failure and evaluation for bilateral pulmonary transplantation. Three years later, he developed bilateral pneumonia requiring mechanical ventilation. When refractory hypoxemia ensued, he had to be placed on extracorporeal veno-venous membrane oxygenation assistance (VV ECMO). Under these conditions he was transferred to our center and included in the waiting list for a bilateral pulmonary transplantation. Forty-eight hours after admission, and due to intense polyuria, central diabetes insipidus was diagnosed. In this clinical context, the presence of cutaneous lesions on the scalp were reconsidered and biopsied under the presumption of possible LCH, with pathology analysis confirming the diagnosis. He continued to be assisted with VV ECMO for 66 more days as a bridge to transplantation, developing multi-organ failure and passing away before a donor organ was available. The diagnosis of LCH should be considered in any adult patient with bullous emphysema of undetermined cause. Given the possibility of early therapeutic interventions, the search for its clinical associations (such as diabetes insipidus and/or skin lesions) should be a systematic part of the etiologic workup. The availability of skin specimens to reach a diagnosis make its thorough search an important part of the diagnostic approach.Fil: Klein, Francisco R.. Fundación Favaloro; ArgentinaFil: Klein, Julia. Sheba Medical Center; IsraelFil: Otalora Lozano, Diego. Fundación Favaloro; ArgentinaFil: Vigliano, Carlos. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Medicina Traslacional, Trasplante y Bioingeniería. Fundación Favaloro. Instituto de Medicina Traslacional, Trasplante y Bioingeniería; ArgentinaSpringer2024-02info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/256986Klein, Francisco R.; Klein, Julia; Otalora Lozano, Diego; Vigliano, Carlos; Late Diagnosis of Langerhans Cell Histiocytosis by Skin Biopsy in a Lung Transplant Candidate Patient; Springer; Cureus; 2024; 2-2024; 1-72168-8184CONICET DigitalCONICETenginfo:eu-repo/semantics/altIdentifier/url/https://www.cureus.com/articles/229439-late-diagnosis-of-langerhans-cell-histiocytosis-by-skin-biopsy-in-a-lung-transplant-candidate-patientinfo:eu-repo/semantics/altIdentifier/doi/10.7759/cureus.55226info:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-09-03T09:47:59Zoai:ri.conicet.gov.ar:11336/256986instacron:CONICETInstitucionalhttp://ri.conicet.gov.ar/Organismo científico-tecnológicoNo correspondehttp://ri.conicet.gov.ar/oai/requestdasensio@conicet.gov.ar; lcarlino@conicet.gov.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:34982025-09-03 09:47:59.808CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv Late Diagnosis of Langerhans Cell Histiocytosis by Skin Biopsy in a Lung Transplant Candidate Patient
title Late Diagnosis of Langerhans Cell Histiocytosis by Skin Biopsy in a Lung Transplant Candidate Patient
spellingShingle Late Diagnosis of Langerhans Cell Histiocytosis by Skin Biopsy in a Lung Transplant Candidate Patient
Klein, Francisco R.
LANGERHANS CELL HISTIOCYTOSIS
SKIN BIOPSY
LUNG TRANSPLANTATION
ACUTE RESPIRATORY FAILURE
title_short Late Diagnosis of Langerhans Cell Histiocytosis by Skin Biopsy in a Lung Transplant Candidate Patient
title_full Late Diagnosis of Langerhans Cell Histiocytosis by Skin Biopsy in a Lung Transplant Candidate Patient
title_fullStr Late Diagnosis of Langerhans Cell Histiocytosis by Skin Biopsy in a Lung Transplant Candidate Patient
title_full_unstemmed Late Diagnosis of Langerhans Cell Histiocytosis by Skin Biopsy in a Lung Transplant Candidate Patient
title_sort Late Diagnosis of Langerhans Cell Histiocytosis by Skin Biopsy in a Lung Transplant Candidate Patient
dc.creator.none.fl_str_mv Klein, Francisco R.
Klein, Julia
Otalora Lozano, Diego
Vigliano, Carlos
author Klein, Francisco R.
author_facet Klein, Francisco R.
Klein, Julia
Otalora Lozano, Diego
Vigliano, Carlos
author_role author
author2 Klein, Julia
Otalora Lozano, Diego
Vigliano, Carlos
author2_role author
author
author
dc.subject.none.fl_str_mv LANGERHANS CELL HISTIOCYTOSIS
SKIN BIOPSY
LUNG TRANSPLANTATION
ACUTE RESPIRATORY FAILURE
topic LANGERHANS CELL HISTIOCYTOSIS
SKIN BIOPSY
LUNG TRANSPLANTATION
ACUTE RESPIRATORY FAILURE
purl_subject.fl_str_mv https://purl.org/becyt/ford/3.2
https://purl.org/becyt/ford/3
https://purl.org/becyt/ford/3.2
https://purl.org/becyt/ford/3
https://purl.org/becyt/ford/3.1
https://purl.org/becyt/ford/3
dc.description.none.fl_txt_mv Langerhans cell histiocytosis (LCH) is a rare disease. In the adult population, isolated pulmonary presentation predominates. The course of the disease can be variable, ranging from mild and self-limited cases to potentially fatal respiratory manifestations, with some individuals requiring lung transplantation. A 20-year-old patient started 3 years before his admission with progressive dyspnea leading to a diagnosis of bullous emphysema of undetermined cause, which evolved to respiratory failure and evaluation for bilateral pulmonary transplantation. Three years later, he developed bilateral pneumonia requiring mechanical ventilation. When refractory hypoxemia ensued, he had to be placed on extracorporeal veno-venous membrane oxygenation assistance (VV ECMO). Under these conditions he was transferred to our center and included in the waiting list for a bilateral pulmonary transplantation. Forty-eight hours after admission, and due to intense polyuria, central diabetes insipidus was diagnosed. In this clinical context, the presence of cutaneous lesions on the scalp were reconsidered and biopsied under the presumption of possible LCH, with pathology analysis confirming the diagnosis. He continued to be assisted with VV ECMO for 66 more days as a bridge to transplantation, developing multi-organ failure and passing away before a donor organ was available. The diagnosis of LCH should be considered in any adult patient with bullous emphysema of undetermined cause. Given the possibility of early therapeutic interventions, the search for its clinical associations (such as diabetes insipidus and/or skin lesions) should be a systematic part of the etiologic workup. The availability of skin specimens to reach a diagnosis make its thorough search an important part of the diagnostic approach.
Fil: Klein, Francisco R.. Fundación Favaloro; Argentina
Fil: Klein, Julia. Sheba Medical Center; Israel
Fil: Otalora Lozano, Diego. Fundación Favaloro; Argentina
Fil: Vigliano, Carlos. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Medicina Traslacional, Trasplante y Bioingeniería. Fundación Favaloro. Instituto de Medicina Traslacional, Trasplante y Bioingeniería; Argentina
description Langerhans cell histiocytosis (LCH) is a rare disease. In the adult population, isolated pulmonary presentation predominates. The course of the disease can be variable, ranging from mild and self-limited cases to potentially fatal respiratory manifestations, with some individuals requiring lung transplantation. A 20-year-old patient started 3 years before his admission with progressive dyspnea leading to a diagnosis of bullous emphysema of undetermined cause, which evolved to respiratory failure and evaluation for bilateral pulmonary transplantation. Three years later, he developed bilateral pneumonia requiring mechanical ventilation. When refractory hypoxemia ensued, he had to be placed on extracorporeal veno-venous membrane oxygenation assistance (VV ECMO). Under these conditions he was transferred to our center and included in the waiting list for a bilateral pulmonary transplantation. Forty-eight hours after admission, and due to intense polyuria, central diabetes insipidus was diagnosed. In this clinical context, the presence of cutaneous lesions on the scalp were reconsidered and biopsied under the presumption of possible LCH, with pathology analysis confirming the diagnosis. He continued to be assisted with VV ECMO for 66 more days as a bridge to transplantation, developing multi-organ failure and passing away before a donor organ was available. The diagnosis of LCH should be considered in any adult patient with bullous emphysema of undetermined cause. Given the possibility of early therapeutic interventions, the search for its clinical associations (such as diabetes insipidus and/or skin lesions) should be a systematic part of the etiologic workup. The availability of skin specimens to reach a diagnosis make its thorough search an important part of the diagnostic approach.
publishDate 2024
dc.date.none.fl_str_mv 2024-02
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
http://purl.org/coar/resource_type/c_6501
info:ar-repo/semantics/articulo
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/11336/256986
Klein, Francisco R.; Klein, Julia; Otalora Lozano, Diego; Vigliano, Carlos; Late Diagnosis of Langerhans Cell Histiocytosis by Skin Biopsy in a Lung Transplant Candidate Patient; Springer; Cureus; 2024; 2-2024; 1-7
2168-8184
CONICET Digital
CONICET
url http://hdl.handle.net/11336/256986
identifier_str_mv Klein, Francisco R.; Klein, Julia; Otalora Lozano, Diego; Vigliano, Carlos; Late Diagnosis of Langerhans Cell Histiocytosis by Skin Biopsy in a Lung Transplant Candidate Patient; Springer; Cureus; 2024; 2-2024; 1-7
2168-8184
CONICET Digital
CONICET
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/url/https://www.cureus.com/articles/229439-late-diagnosis-of-langerhans-cell-histiocytosis-by-skin-biopsy-in-a-lung-transplant-candidate-patient
info:eu-repo/semantics/altIdentifier/doi/10.7759/cureus.55226
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
https://creativecommons.org/licenses/by/2.5/ar/
eu_rights_str_mv openAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by/2.5/ar/
dc.format.none.fl_str_mv application/pdf
application/pdf
dc.publisher.none.fl_str_mv Springer
publisher.none.fl_str_mv Springer
dc.source.none.fl_str_mv reponame:CONICET Digital (CONICET)
instname:Consejo Nacional de Investigaciones Científicas y Técnicas
reponame_str CONICET Digital (CONICET)
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instname_str Consejo Nacional de Investigaciones Científicas y Técnicas
repository.name.fl_str_mv CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicas
repository.mail.fl_str_mv dasensio@conicet.gov.ar; lcarlino@conicet.gov.ar
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