A Novel and Likely Inherited Lymphoproliferative Disease in British Shorthair Kittens

Vet Pathol. 2015 Nov;52(6):1176-82. doi: 10.1177/0300985815586224. Epub 2015 Jun 3.


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.

Keywords: PCR for antigen receptor rearrangement; apoptosis; autoimmune lymphoproliferative syndrome (ALPS); cat; genetic predisposition to disease; lymphadenopathy; lymphoma; lymphoproliferative disorders.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cat Diseases / genetics*
  • Cat Diseases / pathology
  • Cats
  • Female
  • Genetic Predisposition to Disease
  • Lymphadenopathy
  • Lymphoma, T-Cell / genetics
  • Lymphoma, T-Cell / pathology
  • Lymphoma, T-Cell / veterinary*
  • Lymphoproliferative Disorders / genetics
  • Lymphoproliferative Disorders / pathology
  • Lymphoproliferative Disorders / veterinary*
  • Male
  • Splenomegaly