Dramatic response to nivolumab in xeroderma pigmentosum skin tumor.

We report the case of a 6-year-old female with xeroderma pigmentosum (XP) who developed a nonoperable scalp tumor, treated with anti-programmed cell death protein 1 (anti-PD-1) therapy (nivolumab). She presented with a sarcomatoid carcinoma of the scalp with bone lysis as well as vascular and meningeal contact. Nivolumab was initiated because it has emerged as a promising immunotherapy. We observed a dramatic tumor response with excellent tolerance. However, while on nivolumab therapy she developed two large skin melanomas and several squamous cell carcinomas, which have been resected. These results demonstrate that cancer immunotherapy in patients with XP can be impressive but complex and warrants further investigation.

Vaccination against influenza and pneumococcal infections in patients with autoimmune disorders under biological therapy: Coverage and attitudes in patients and physicians.

BACKGROUND: Patients under biological therapy for auto-immune disease are considered immunosuppressed and several recent recommendations highlight the need for vaccination against influenza and pneumococcal infections. The aims of this study were to evaluate influenza and pneumococcal vaccine coverage among patients receiving biological therapy and identify factors associated with vaccine uptake within this population. METHODS: A retrospective cross-sectional study was performed in adult patients attending hospitals for an auto-immune/inflammatory disease and treated with biological therapy. Vaccine uptake was evidenced from patient's medical records or from their pharmacist's records. Questionnaires about attitudes and knowledge regarding vaccinations were administered to patients and their physicians. Multivariable logistic regression was used to determine factors significantly associated with influenza and pneumococcal vaccine receipt. RESULTS: A total of 208 patients were included: 52% female and mean age 50.6 (± 14.7) years. Among them 173 completed the questionnaire while 72 physicians replied. Underlying inflammatory diseases were rheumatisms (46%), bowel diseases (31%) and skin diseases (23%). Vaccine uptake was 28% for influenza, 48% for pneumococcus and 22% received both vaccines. Main factors associated to positive uptake were receiving a prescription from a physician, as well as having a good knowledge of vaccines. Factors limiting vaccination were a negative attitude toward vaccines in general, and belonging to the group of inflammatory bowel diseases. CONCLUSIONS: Vaccine coverage for influenza and pneumococcal infections are low in the patients under biologics for auto-immune/inflammatory disease. Health policies should reinforce information and promotion of these vaccines among these patients but also the prescribers.