Isolated Pulmonary Langerhans Cell Histiocytosis

Varkki, Sneha; Tergestina, Mintoo; Bhonsle, Vaishali Sharad; Moses, Prabhakar D.; Mathai, John; Korula, Sophy

doi:10.1007/s12098-012-0866-x

Cited by 12 publications

(14 citation statements)

References 8 publications

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“…Interestingly, alveolar histiocytosis has been demonstrated in rats after exposure in aqueous dispersion of resins that are normally contained in hair‐spray formulations . Involvement of lungs in LCH may be an adverse prognostic factor due to the irreversible parenchymal destruction, characterized by fibrosis, scarring, and cyst formation, that follows the infiltration of Langerhans cells (LCs) and other inflammatory cells in the early stages of the disease . Airway‐centered inflammatory destruction may be responsible for the injurious alterations to lung structures .…”

Section: Discussionmentioning

confidence: 99%

“…Langerhans cell histiocytosis (LCH) is a rare disease in childhood, occurring in approximately 1.0–4.6 per million children <14 years old. Pulmonary involvement in the setting of multiple multisystem‐LCH is seen at diagnosis in approximately 25% of the cases, but isolated pulmonary LCH is extremely rare in children, accounting for <1% of all pediatric LCH cases . In contrast with other forms of single system‐LCH, pulmonary LCH may induce permanent pulmonary dysfunction and death .…”

Section: Introductionmentioning

confidence: 99%

“…Pulmonary involvement in the setting of multiple multisystem-LCH is seen at diagnosis in approximately 25% of the cases, but isolated pulmonary LCH is extremely rare in children, accounting for <1% of all pediatric LCH cases. [1][2][3] In contrast with other forms of single system-LCH, pulmonary LCH may induce permanent pulmonary dysfunction and death. [1][2][3] As shown in this report, organ dysfunction at diagnosis with no response in the first few weeks of treatment are negative prognostic factors.…”

Section: Introductionmentioning

confidence: 99%

“…[1][2][3] In contrast with other forms of single system-LCH, pulmonary LCH may induce permanent pulmonary dysfunction and death. [1][2][3] As shown in this report, organ dysfunction at diagnosis with no response in the first few weeks of treatment are negative prognostic factors. The speed of the disease progression raised questions on the pathogenetic mechanisms involved, while the successful outcome of long-term extracorporeal membrane oxygenation (ECMO) proved feasible and effective support as a bridge to lung transplantation (LTx).…”

Section: Introductionmentioning

confidence: 99%

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Long-Term Extracorporeal Membrane Oxygenation as Bridging Strategies to Lung Transplantation in Rapidly Devastating Isolated Langerhans Cell Histiocytosis

Sacco

Moscatelli

Conte

et al. 2016

Pediatr Blood Cancer

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Isolated pulmonary involvement in pediatric Langerhans cell histiocytosis (LCH) is extremely rare. While the multisystem-LCH course varies from spontaneous remission to rapid deterioration with lethal outcome, single system involvement is generally associated with favorable prognosis. A child with isolated pulmonary LCH had an extremely rapid progression leading to respiratory failure, despite treatment with prednisone and vinblastine. Since lung hyperinflation and cystic degeneration contraindicated conventional mechanical ventilation, extracorporeal membrane oxygenation (ECMO) was chosen for 50 days as a bridge to lung transplantation. The mechanisms involved in disease progression and the usefulness of long-term ECMO are discussed.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Long-Term Extracorporeal Membrane Oxygenation as Bridging Strategies to Lung Transplantation in Rapidly Devastating Isolated Langerhans Cell Histiocytosis

Sacco

Moscatelli

Conte

et al. 2016

Pediatr Blood Cancer

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“…Procesos inflamatorios y enfermedades del tejido conectivo: histiocitosis de células de Langerhans, enfermedad intersticial pulmonar caracterizada por bullas, quistes o nódulos con o sin cavitación, que se presentan en la vía aérea de pequeño calibre, con tracción de áreas de enfisema y distensión pulmonar, que aumentan el riesgo de neumotórax. 10,13 En una muestra de 102 pacientes con HCL pulmonar confirmado histológicamente, el 16% (n= 16) presentó uno o más episodios de neumotórax. De estos, en 11 pacientes (69%), la manifestación inicial fueron neumotórax espontáneos, con recurrencia del 4 al 17% y mortalidad del 16%, relacionada con neumotórax recurrente.…”

Section: Figura 2 Tomografía Computada De Tórax Imágenes De Aspectounclassified

Neumotórax bilateral espontáneo como forma de presentación de histiocitosis de células de Langerhans

2014

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ResumenLa histiocitosis de células de Langerhans pulmonar es una patología intersticial en la que existe un acúmulo de células histiocíticas específicas a nivel pulmonar. El neumotórax espontáneo es una complicación reconocida de histiocitosis de células de Langerhans pulmonar y es secundario a la destrucción del parénquima pulmonar con cambios quísticos asociados. Reportamos el caso de un niño de 2 años con neumotórax espontáneo bilateral recurrente, con una tomografía axial computada de tórax con infiltrado intersticial, fibrosis, lesiones quísticas e imágenes bullosas. El diagnóstico fue establecido por examen histológico e inmunohistoquímica de tejido de biopsia pulmonar con anticuerpos CD1 y S100 positivos. El niño recibió tratamiento con prednisona y etopósido, con buena respuesta clínica y tomográfica. Palabras clave: neumotórax espontáneo, histiocitosis de células de Langerhans pulmonar. ABsTRACTPulmonary Langerhans cell histiocytosis is an interstitial lung disease that results from the accumulation of specific histiocytic cells in the lung. Spontaneous pneumothorax is a recognized feature of pulmonary Langerhans cell histiocytosis and results from destruction of lung parenchyma with associated cystic changes. We report on a 2-year-old boy with recurrent bilateral spontaneous pneumothorax; a computed tomography scan showed marked interstitial changes, fibrosis, cystic spaces and bilateral bullae. The diagnosis was confirmed by the histology and the immunohistochemistry examination of the pulmonary biopsy with CD1 and S100 positive antibodies. The child was treated with prednisone and etoposide, and had a good clinical response and favorable changes in the second thoracic CT scan. Bilateral spontaneous pneumothorax as a setting of Langerhans cell histiocytosis InTRoduCCIónLa histiocitosis de células de Langerhans (HCL) comprende un grupo de enfermedades con diferente curso clínico. Previamente, se incluían en este grupo el granuloma eosinofílico, la enfermedad de Hand-Schüller-Christian y la enfermedad de Letterer-Siwe. Luego, se agruparon como histiocitosis X, que, en 1987, fue clasificada como histiocitosis de células de Langerhans. 1-2La HCL tiene una presentación variable: puede afectar a un solo órgano, como hueso, piel, nódulos linfáticos, pulmón y glándula pituitaria, o ser de afectación multisistémica, que incluye el sistema hematopoyético, hígado, bazo y pulmones, y que tiene una mortalidad significativamente alta. 3-4La afectación pulmonar por HCL es muy rara en niños; contrariamente, en adultos, se reporta entre un 90 y un 100% de asociación entre HCL pulmonar y antecedentes de tabaquismo. 5-6Se desconoce la prevalencia de HCL pulmonar. Informes de una serie de biopsias pulmonares en pacientes adultos con patología pulmonar intersticial reportan una frecuencia del 5% de HCL pulmonar.7 En una serie de 1382 pacientes adultos con patología pulmonar difusa, en 91 (6,6%), se estableció el diagnóstico de HCL pulmonar. 8Los pacientes con HCL pulmonar presentan síntomas respiratorios inespecíficos, c...

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Chronic cough and weight loss in an adolescent marijuana smoker

Onorato

Shapiro

Griffin

et al. 2022

SAGE Open Medical Case Reports

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Marijuana is the most widely used illicit drug in the United States. As marijuana becomes legalized in more states and its use increases among adolescents, pediatricians must be aware of the impact of marijuana on pediatric health. Marijuana smoking as well as cigarette smoking has been associated with numerous lung diseases, including chronic bronchitis and bullous lung diseases. This case report postulates that regular marijuana smoking may be associated with pulmonary Langerhans cell histiocytosis, a severe lung disease that lacks definitive treatment and can cause respiratory failure. Given the potential risk of life-threatening lung diseases, pediatricians must screen adolescents with respiratory symptoms for marijuana use. In addition, this case underscores the need for further research and improved understanding of the relationship between marijuana smoking and lung disease.

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Isolated Pulmonary Langerhans Cell Histiocytosis

Cited by 12 publications

References 8 publications

Long-Term Extracorporeal Membrane Oxygenation as Bridging Strategies to Lung Transplantation in Rapidly Devastating Isolated Langerhans Cell Histiocytosis

Long-Term Extracorporeal Membrane Oxygenation as Bridging Strategies to Lung Transplantation in Rapidly Devastating Isolated Langerhans Cell Histiocytosis

Neumotórax bilateral espontáneo como forma de presentación de histiocitosis de células de Langerhans

Chronic cough and weight loss in an adolescent marijuana smoker

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