[Efficacy of rituximab in lymphomatoid granulomatosis]. / Efficacité du Rituximab au cours d'une granulomatose lymphomatoïde.

INTRODUCTION: Lymphomatoid Granulomatosis is a rare and serious disease, now considered to be a B-cell lymphoma, which is frequently associated with Epstein-Barr virus infection. There is no consensus on treatment, which is usually based on steroid therapy, either alone or combined with cyclophosphamide and combination chemotherapy. CASE REPORT: We report the case of an asymptomatic patient diagnosed after the incidental discovery of bilateral nodular opacities on their chest x-ray. Physical examination and bronchoscopy were normal. The diagnosis of Lymphomatoid Granulomatosis was made on the basis of surgical lung biopsy. Immunohistochemical studies confirmed the B phenotype of the lymphoma with the identification of atypical large CD 20 positive cells. In situ hybridisation confirmed the presence of EBV. In this case the course of the disease was slow. Treatment with anti CD 20 monoclonal antibodies (rituximab) led initially to a reduction in parenchymal abnormalities and mediastinal adenopathy. CONCLUSION: This treatment, recently used in Lymphomatoid Granulomatosis with pulmonary involvement, has shown promising results. Rituximab can be used in combination chemotherapy as standard treatment for aggressive B-cell lymphoma.

[Treatment of tracheobronchomegaly using the Freitag prosthesis]. / Traitement de la trachéobronchomégalie par prothèse de Freitag.

Tracheobronchomegaly (TBM) is a rare disorder. It is characterised by a dilatation of the trachea and subsequent bronchial divisions associated with a dynamic pathology, in particular a cough which explains the symptomatology of the patients. We report the observation of a patient suffering from TBM whose clinical progress rapidly improved after the insertion of a Freitag prosthesis. This 60-year-old male presented with a year's history of chronic cough which was painful, cavernous and had been incapacitating associated with moderate dyspnoea of effort. Bronchial endoscopy showed dyskinesia extending almost totally throughout the tracheobronchial tree with complete expiratory collapse. The FEV1, the Vital Capacity and the TLC were subnormal and the PEF was 57% of the predicted. The total airways resistance (RAW) was elevated (306% of the predicted), the arterial blood gases were normal. A CT scan showed a deformed trachea with increased diameter. A tracheobronchial prosthesis of Freitag or Dynamic stent (Rüsch) was inserted with the help of a rigid bronchoscope. One month later the cough had regressed and the effort dyspnoea had disappeared. One year later the clinical improvement persisted with very good tolerance of the prosthesis with normal spirometry despite the persistence of an elevated RAW. Despite a recoil which is still imperfect the tracheobronchial endoprosthesis seems to be a useful treatment for forms of TBM which are potentially progressive.