Aberdein D, Munday J S, Fairley R A, Vernau W, Thompson K G
Department of Pathobiology, Institute of Veterinary, Animal and Biomedical Sciences, Massey University, Palmerston North, New Zealand
Department of Pathobiology, Institute of Veterinary, Animal and Biomedical Sciences, Massey University, Palmerston North, New Zealand.
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.
在多只亲缘关系密切的英国短毛猫幼崽中发现了一种不寻常的淋巴增生性疾病，这表明该病具有遗传易感性。受影响的幼猫通常从6周龄开始出现迅速进展且明显的全身性淋巴结病、中度脾肿大以及再生性且可能为溶血性的贫血。显微镜检查结果提示为多中心性T细胞淋巴瘤，但进一步检测发现了一群CD3+/CD4-/CD8-“双阴性”T细胞（DNT细胞）的多克隆群体。这是一种新的疾病表现，与人类疾病自身免疫性淋巴增生综合征（ALPS）相似，后者是一种罕见的遗传性疾病，可导致淋巴增生和自身免疫的多种表现。人类疾病最常见的原因是Fas基因突变导致淋巴细胞凋亡缺陷，目前正在对患病猫的遗传方式和疾病的遗传基础进行进一步研究。
