PhD student: Amira Benattia

Title: Phenotypic characterization and prognosis of adult pulmonary Langerhans cell histiocytosis

Supervisors: Pr Raphaël Porcher, Pr Abdellatif Tazi

Doctoral school: ED 393 Ecole doctorale Pierre Louis de sante publique

Promotion: 2023/2024

Funding: No funding dedicated to the thesis. Salaried activity (hospital practitioner)

Thesis abstract

Langerhans cell histiocytosis (LCH) is a rare cosmopolitan disease of undetermined etiology characterized by infiltration of affected organs by CD1a and/or langerin (CD207) cells derived from dendritic cells. Pulmonary involvement of LCH (PLCH) is most often isolated in adults and affects young smokers or ex-smokers electively. Its natural history is variable and difficult to predict: possible spontaneous remission or after smoking cessation, progression, more rarely, rapid worsening leading to chronic respiratory failure. Identifying patients at risk at the time of diagnosis is a major challenge for patient care. The overall objective of this thesis work is to better characterize the functional, radiological, and biological phenotype of adult PLCH and to identify prognostic factors. The first objective is to identify evolutionary trajectories of FEV1, the factors associated with these profiles, including the study of serum biomarkers, and to assess their association with survival. The second objective is to characterize chest computed tomography abnormalities observed at diagnosis using artificial intelligence tools and to assess their association with prognosis in the same cohort of patients. The third part of the work, more exploratory, aims to evaluate the contribution of a quantitative method compiling images in inspiration and exhalation called “parametric response mapping” in the characterization of lesions and its correlation with respiratory function.


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