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Nor98-like atypical scrapie is a sporadic disease that affects the central nervous system of sheep and goats that, in contrast to classical scrapie, is not generally regarded as naturally transmissible. However, infectivity has been demonstrated via bioassay not only of brain tissue but also of certain peripheral nerves, lymphoid tissues, and muscl...
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Since the beginning prion research has been largely dependent on animal models for deciphering the disease, drug development or prion detection and quantification. Thereby, ethical as well as cost and labour-saving aspects call for alternatives in vitro. Cell models can replace or at least complement animal studies, but their number is still limite...
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... The wether was a second-generation relative of a ewe experimentally inoculated with atypical (Nor98-like) scrapie. 1 The inoculated ewe (the grandparent of the presenting patient) became infected with atypical scrapie but never developed clinical signs despite reaching 7 years of age. First-and second-generation offspring of these ewes, including the presenting patient, except for a rectal temperature of 105.6°F, which was unresponsive to NSAIDs. ...
... The wether described herein was part of a natural transmission study of atypical scrapie. 1 The study included four ewes initially infected with atypical scrapie by inoculation, 32 first-generation offspring, 11 second-generation offspring including this patient, and seven breeding rams. Atypical scrapie is a transmissible spongiform encephalopathy (TSE) or prion disease that primarily affects sheep and goats. ...
B‐cell leukemia is a rare form of hematologic neoplasia in sheep, especially in adult animals. We present a case report of a 5‐year‐old WhiteFace Sheep wether with suspected acute lymphoblastic leukemia. The patient, a second‐generation relative of ewes experimentally inoculated with atypical scrapie, exhibited acute lethargy and loss of appetite. Laboratory investigation revealed marked leukocytosis, lymphocytosis, and abnormal serum chemistry panel results. Microscopic examination of blood and bone marrow smears exhibited a high percentage of large neoplastic cells with lymphoid characteristics. Histopathologic analysis of the spleen, liver, lungs, and other organs confirmed the presence of widespread tissue infiltration by neoplastic cells. Immunohistochemical labeling demonstrated strong intracytoplasmic labeling for CD20, consistent with B‐cell neoplasia. Flow cytometric analysis confirmed the B‐cell lineage of the neoplastic cells. Screening for bovine leukemia virus, which can experimentally cause leukemia in sheep, yielded a negative result. In this case, the diagnosis of B‐cell leukemia was supported by a comprehensive panel of diagnostic evaluations, including cytology, histopathology, immunohistochemistry, and immunophenotyping. This case report highlights the significance of accurate diagnosis and classification of hematologic neoplasia in sheep, emphasizing the need for immunophenotyping to aid in the diagnosis of B‐cell leukemia. It also emphasizes the importance of considering spontaneous leukemia as a differential diagnosis in sheep with lymphoid neoplasia, especially in the absence of circulating infectious diseases.
