Diversity in Neuroanatomical Distribution of Abnormal Prion Protein in Atypical Scrapie

Scrapie is a transmissible spongiform encephalopathy (TSE) in sheep and goats. In recent years, atypical scrapie cases were identified that differed from classical scrapie in the molecular characteristics of the disease-associated pathological prion protein (PrPsc). In this study, we analyze the molecular and neuropathological phenotype of nine Swiss TSE cases in sheep and goats. One sheep was identified as classical scrapie, whereas six sheep, as well as two goats, were classified as atypical scrapie. The latter revealed a uniform electrophoretic mobility pattern of the proteinase K–resistant core fragment of PrPsc distinct from classical scrapie regardless of the genotype, the species, and the neuroanatomical structure. Remarkably different types of neuroanatomical PrPsc distribution were observed in atypical scrapie cases by both western immunoblotting and immunohistochemistry. Our findings indicate that the biodiversity in atypical scrapie is larger than expected and thus impacts on current sampling and testing strategies in small ruminant TSE surveillance

Mislabeled units of umbilical cord blood detected by a quality assurance program at the transplantation center

We instituted procedures to check the identity of cord blood unit provided for transplantation by carrying out ABO and human leukocyte antigen (HLA) typing of the thawed units before transplantation. ABO typing is done using standard techniques. Rapid HLA class I serology is with monoclonal antibody trays (One Lambda Inc) using standard incubations. One mislabeled umbilical cord blood (UCB) unit was detected on the day of intended transplantation by repeat ABO typing of the thawed unit at our transplantation center. Because ABO typing will not detect all labeling errors, the rapid serologic class I HLA typing procedure was done on thawed units just before transplantation for all units without an attached segment. This procedure identified a second mislabeled unit. In a 6-year period, 2 of 871 (0.2%) cord blood units sent to us for transplantation were mislabeled and potentially would have been transplanted incorrectly. This error rate of 1 per 249 (0.4%) patients could have potentially devastating consequences