Switch to pdVWF:pdFVIII concentrate for prophylaxis in a paediatric patient with Type 3 von Willebrand disease: a case report.

OBJECTIVES: To report the long-term prophylaxis management of a child with type 3 von Willebrand disease by switching to Wilate (Octapharma AG), a plasma-derived, double virus-inactivated concentrate of freeze-dried of a 1 to 1 ratio of active Von Willebrand Factor and Factor VIII (pdVWF:pdFVIII) recently marketed as Eqwilate in France. METHODS: This is a case report of 12.6-year-old boy with congenital Type 3 VWD who had a history of frequent bleeds. Prophylaxis started at the age of 38 months with FVIII-poor pdVWF concentrate (Wilfactin, LFB) and FVIII (Wilstart, LFB). Pharmacokinetics and thrombin generation assay were performed. Annualized bleeding rate was derived from the bleeding episodes documented in the medical record during a 24-month period before and after starting pdVWF:pdFVIII concentrate. RESULTS: Both product injections promptly raised the endogenous thrombin potential (ETP). However, the maximal concentration of formed thrombin was higher following pdVWF:pdFVIII injection. Due to a high bleeds frequency and better results regarding FVIII levels and thrombin generation, the prophylaxis regimen was changed to the same dose and frequency of pdVWF:pdFVIII concentrate (42 IU/kg per day, three times a week). During the last 24 months, annualized total, trauma, and spontaneous bleeding rates were 7.5, 4.5, and 3, respectively. These rates decreased to 2, 1.5, and 0.5 respectively during the next two years. The mother reported a marked improvement in the quality of life of his son and hers. CONCLUSION: Switch to pdVWF:pdFVIII concentrate for long-term prophylaxis in a young type 3 VWD patient was safe and effective in reducing bleeds.

Listeria monocytogenes and ocular abscess: an atypical but yet potential association.

PURPOSE: To report a farmer's corneal abscess caused by an unusual pathogen: Listeria monocytogenes fluoroquinolone resistant. METHODS: A 78-year-old farmer presented a central corneal abscess associated with 1-mm hypopyon and decreased visual acuity evolving since 2 weeks. First an antibiotic therapy associating oral ofloxacin and topical ciprofloxacin, vancomycin and ceftazidime was started. Different samples of the abscess were performed and sent to different microbiological laboratories. RESULT: Listeria monocytogenes was isolated after 2 days of culture. Antibiotics sensitivity showed resistance to ciprofloxacin, fosfomycin and fusidic acid. Ceftazidime was changed for gentamicin, and after 1 month of treatment the abscess decreased considerably. CONCLUSION: This case demonstrated that even if Listeria is rarely involved in ocular abscess, it must be evocated for people with risk factors as farmers. This suspicion should lead to an extended incubation to identify the pathogen. The analysis of Listeria resistance is essential to start an efficient therapy.