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
- Institución
- Consejo Nacional de Investigaciones Científicas y Técnicas
- OAI Identificador
- oai:ri.conicet.gov.ar:11336/256986
Ver los metadatos del registro completo
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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) |
collection |
CONICET Digital (CONICET) |
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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1842268895675678720 |
score |
13.13397 |