title: A Novel and Likely Inherited Lymphoproliferative Disease in British Shorthair Kittens.
abstract: An unusual lymphoproliferative disease was identified in multiple closely related British Shorthair (BSH) kittens, suggesting an inherited predisposition to disease. Affected kittens typically developed rapidly progressive and marked generalized lymphadenopathy, moderate splenomegaly, and regenerative and likely hemolytic anemia from 6 weeks of age. Microscopic findings were suggestive of multicentric T-cell lymphoma, but additional testing revealed a polyclonal population of CD3+/CD4-/CD8- "double negative" T cells (DNT cells). This is a novel disease presentation with similarities to the human disorder autoimmune lymphoproliferative syndrome (ALPS), a rare inherited disease causing lymphoproliferation and variable manifestations of autoimmunity. The human disease is most commonly due to the presence of Fas gene mutations causing defective lymphocyte apoptosis, and further investigations of both the mode of inheritance and genetic basis for disease in affected cats are currently in progress.
authors.affiliation: Department of Pathobiology, Institute of Veterinary, Animal and Biomedical Sciences, Massey University, Palmerston North, New Zealand D.Aberdein@massey.ac.nz.
authors.forename: D
authors.initials: D
authors.lastname: Aberdein
authors.affiliation: Department of Pathobiology, Institute of Veterinary, Animal and Biomedical Sciences, Massey University, Palmerston North, New Zealand.
authors.forename: J S
authors.initials: JS
authors.lastname: Munday
authors.affiliation: Gribbles Veterinary Diagnostic Laboratory, Christchurch, New Zealand.
authors.forename: R A
authors.initials: RA
authors.lastname: Fairley
authors.affiliation: Department of Pathology, Microbiology, and Immunology, School of Veterinary Medicine, University of California, Davis, CA, USA.
authors.forename: W
authors.initials: W
authors.lastname: Vernau
authors.affiliation: Department of Pathobiology, Institute of Veterinary, Animal and Biomedical Sciences, Massey University, Palmerston North, New Zealand.
authors.forename: K G
authors.initials: KG
authors.lastname: Thompson
mesh_terms: D000818:Animals; D002371:Cat Diseases / Q000235:genetics* / Q000473:pathology; D002415:Cats; D005260:Female; D020022:Genetic Predisposition to Disease; D000072281:Lymphadenopathy; D016399:Lymphoma, T-Cell / Q000235:genetics / Q000473:pathology / Q000662:veterinary*; D008232:Lymphoproliferative Disorders / Q000235:genetics / Q000473:pathology / Q000662:veterinary*; D008297:Male; D013163:Splenomegaly
keywords: PCR for antigen receptor rearrangement; apoptosis; autoimmune lymphoproliferative syndrome (ALPS); cat; genetic predisposition to disease; lymphadenopathy; lymphoma; lymphoproliferative disorders