[Postmarketing surveillance study on the use of topotecan in small-cell lung cancer. Use of topotecan in a pneumology university department]. / Étude post-autorisation de mise sur le marché sur le topotécan dans le cancer bronchique à petites cellules. Utilisation du topotécan dans un service de pneumologie universitaire.

BACKGROUND: Lung cancer is the leading cause of cancer related death in France and Europe. Small-cell lung carcinoma (SCLC) represents 15-20% of cases. International standards of care recommend the use of first-line chemotherapy, which has a high response rate. However, tumour recurrence occurs after a variable disease-free period. If the first-line treatment cannot be repeated during the relapse, intravenous topotecan may be used according to its market authorization (MA). METHODS: We report the first French postmarketing surveillance study on the use of topotecan in the SCLC. RESULTS: Between August 2005 to December 2009, 26 SCLC patients received at least one cycle of intravenous topotecan in our department. All patients were treated in accordance with MA. Seventeen patients received this treatment as second line, as in the MA study, while nine patients where treated beyond the second line. In the first group, we showed that the anti-tumour efficiency of topotecan is similar to that observed in the MA study (24% response) while grade 3 to 4 haematological toxicities were less frequent. In the second group of patients, who could not have been included in the MA study, the tumour response rate was equal to 11% without an increase in severe toxicity compared to the first group. Specific or overall survival was not different between the two groups. CONCLUSION: Our study provides original public health data on the target population, the anti-tumor efficiency and the toxicity of topotecan in the treatment of SCLC.

[Type B Niemann Pick disease: clinical description of three patients in a same family]. / Maladie de Niemann-Pick de type B: description clinique de trois cas familiaux.

INTRODUCTION: The Niemann Pick disease type B is a rare deficiency in sphingomyelinase activity, autosomal recessively inherited. CASE REPORTS: We report three patients (two men, one woman) of the same family, who showed pulmonary and hepatosplenic lesions, usually present in the disease but also adrenal gland lesions confirmed by tomodensitometry. CONCLUSION: The current treatment of Niemann Pick disease is purely symptomatic awaiting the use of enzymatic replacement therapy which has been successfully experimented in animal model.