Manifestations pulmonaires de l’histiocytose langerhansienne

Obert, J.; Tazi, Abdellatif

doi:10.1016/j.rmr.2015.01.005

Cited by 11 publications

(3 citation statements)

References 108 publications

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“…Dynamic volumes (FEV 1 , FVC) were decreased in patients with impaired aerobic capacity, while static volumes (TLC, FRC) were similar in patients with and without impaired aerobic capacity. This functional profile—commonly called “pseudorestriction”—is typically observed in bronchiolar diseases, in which decreased pulmonary compliance due to cellular infiltration of the lung decreases dynamic volumes, while air trapping increases RV leading to globally normal static volumes [21]. Decreased FEV 1 and FVC were unlikely due to airway obstruction, as PC2 did not correlate with FEV 1 /FVC.…”

Section: Discussionmentioning

confidence: 99%

Physiologic Determinants of Exercise Capacity in Pulmonary Langerhans Cell Histiocytosis: A Multidimensional Analysis

Rolland‐Debord

Fry²,

Giovannelli³

et al. 2017

PLoS ONE

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BackgroundReduced exercise capacity severely impacts quality of life in pulmonary Langerhans cell histiocytosis. Ascertaining mechanisms that impair exercise capacity is necessary to identify targets for symptomatic treatments.MethodsDyspnea, pulmonary function tests and cardiopulmonary exercise test were analysed in 62 study participants. Data were compared between subjects with impaired and normal aerobic capacity (V’O2 peak less than 84% versus 84% predicted or more). Data were reduced using a principal component analysis. Multivariate analysis included V’O2 peak as the dependent variable and principal components as covariates.ResultsV’O2 peak was reduced in 44 subjects (71%). Subjects with impaired aerobic capacity presented: (i) decreased FEV1, FVC, FEV1/FVC, DLCO and DLCO/VA and increased AaDO2, (ii) increased ventilatory equivalents at ventilatory threshold, VD/VT peak, AaDO2 peak and PaCO2 peak and decreased ventilatory reserve and PaO2 peak. There was no difference between groups in dyspnea scores. Principal component analysis extracted 4 principal components interpreted as follows: PC1: gas exchange; PC2: “pseudorestriction”; PC3: exercise-induced hyperpnea; PC4: air trapping. Multivariate analysis explained 65% of V’O2 peak. The 4 principal components were independently associated with V’O2 peak (βcoefficients: PC1: 9.3 [4.6; 14], PC2: 7.5 [3; 11.9], PC3: -5.3 [-9.6;-1.], PC4: -9.8 [-14,9;-4.7]).ConclusionImpaired exercise capacity is frequent in pulmonary Langerhans cell histiocytosis. It is mainly caused by pulmonary changes but is not associated with increased dyspnea intensity. Therefore, treating the lung represents a relevant approach for improving exercise capacity, even in patients experiencing mild dyspnea.

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Section: Discussionmentioning

confidence: 99%

Physiologic Determinants of Exercise Capacity in Pulmonary Langerhans Cell Histiocytosis: A Multidimensional Analysis

Rolland‐Debord

Fry²,

Giovannelli³

et al. 2017

PLoS ONE

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“…L’anomalie la plus fréquemment retrouvée en pléthysmographie est un piégeage aérique dans 45% des cas, suivi d’un trouble ventilatoire obstructif dans 30% des cas puis d’un restrictif dans 6% des cas. Une baisse de la DLCO serait notée chez 80% des patients [ 3 ]. Ceci concorde avec les résultats de la pléthysmographie de notre patient où une augmentation du volume résiduel (VR) et un rapport VR/CPT à 234% et 227% respectivement étaient observés.…”

Section: Discussionunclassified

“…Leur nature langerhansienne est confirmée par immunohistochimie grâce à leur expression de l’antigène membranaire CD1a ou de la Langerine (CD207). L’aspect du granulome varie en fonction du stade évolutif et du tissu concerné [ 3 ].…”

Section: Discussionunclassified

Histiocytose langerhansienne pulmonaire révélée par un pneumothorax: à propos d’un cas

Sajiai¹,

Rachidi²,

Serhane³

et al. 2016

Pan Afr Med J

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L’histiocytose langerhansienne est une affection rare d’étiologie inconnue caractérisée par une infiltration d’un ou plusieurs organes, par des cellules de type Langerhans. Elle a une présentation clinique polymorphe. Nous rapportons le cas de Mr R.Y, âgé de 22 ans, tabagique à 8 PA, admis pour pneumothorax total spontané droit. Un drainage thoracique a été réalisé avec bonne évolution. La TDM thoracique de contrôle a objectivé de multiples formations kystiques diffuses prédominant aux lobes supérieurs. Un bilan a été réalisé à la recherche d’une histiocytose systémique mais s’est révélé négatif. L’évolution était marquée par la récidive du pneumothorax, le recours à une pleurodèse et la réalisation d’une biopsie pulmonaire qui a confirmé le diagnostic. Le diagnostic de l’HistiocytoseLangerhansienne doit être évoqué devant un pneumothorax sur poumon kystique. Le diagnostic est aisé devant un tableau clinique et radiologique évocateur. Néanmoins, les possibilités thérapeutiques restent limitées et la récidive du pneumothorax est fréquente.

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Langerhans cell histiocytosis with lung involvement in isolation and multisystem disease: Staging, natural history, and comparative survival

Hazim

Ruan

et al. 2021

American J Hematol

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Langerhans cell histiocytosis (LCH) is a histiocytic neoplasm that can involve the lungs as single system (LCH-SSL) or multisystem disease (LCH-MSL). The role of fullbody radiographic staging to determine whether patients have LCH-SSL or LCH-MSL is unclear. Long-term outcomes of LCH-SSL versus LCH-MSL and multisystem without lung involvement (LCH-MSNL) are unknown. A retrospective study of adult LCH patients seen at our center from January 2000 to 2020 was performed. In Part 1, we addressed utility of whole-body staging imaging among those presenting with isolated pulmonary signs or symptoms. Staging was defined as fluorodeoxyglucose positron emission tomography-computed tomography (CT) or whole-body CT obtained within 3 months of diagnosis. In Part 2, we examined the frequency of developing extra-pulmonary disease over time and mortality in patients with LCH-SSL. In Part 3, we compared the overall survival of LCH-SSL, LCH-MSL, and LCH-MSNL. Part 1: 240 patients with LCH were identified. A total of 112 (47%) had pulmonary signs or symptoms at presentation. Thirty-four (30%) underwent radiographic staging and only one showed evidence of extra-pulmonary disease. Part 2: 108 (45%) were LCH-SSL. Median follow-up duration of 4.5 years (95% confidence interval [

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Manifestations pulmonaires de l’histiocytose langerhansienne

Cited by 11 publications

References 108 publications

Physiologic Determinants of Exercise Capacity in Pulmonary Langerhans Cell Histiocytosis: A Multidimensional Analysis

Physiologic Determinants of Exercise Capacity in Pulmonary Langerhans Cell Histiocytosis: A Multidimensional Analysis

Histiocytose langerhansienne pulmonaire révélée par un pneumothorax: à propos d’un cas

Langerhans cell histiocytosis with lung involvement in isolation and multisystem disease: Staging, natural history, and comparative survival

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