Weiss Douglas J, Henson Michael
Department of Veterinary Biomedical Sciences, College of Veterinary Medicine, University of Minnesota, St. Paul, MN, USA.
Vet Clin Pathol. 2007 Dec;36(4):373-5. doi: 10.1111/j.1939-165x.2007.tb00445.x.
A 3-year-old Irish Wolfhound was evaluated because of acute onset of lethargy and fever. Severe neutropenia (0/microL; reference interval 2500-11,200/microL) was associated with granulocyte aplasia in the bone marrow (myeloid:erythroid ratio, 0.009:1). Antineutrophil antibodies were assessed by an indirect immunofluorescence assay using flow cytometry. When normal canine leukocytes were incubated with the patient's serum and anti-IgG, a marked shift was observed in the forward-angle light scatter of the neutrophil population, and the monocyte cluster disappeared, possibly the result of fragmentation or lysis. Both neutrophil fluorescence intensity (309 +/- 11 median channel units [MCU], control values 107-152 MCU) and the percentage of neutrophils with increased fluorescence intensity (61 +/- 5%, control values 3.8-13.7%) were increased in the patient's serum, consistent with the presence of antineutrophil antibodies. Repeated episodes of neutropenia occurred while treatment with steroidal and nonsteroidal immunosuppressive therapy was initiated and modified. The neutrophil count eventually stabilized in the low-normal range, and the dog was maintained for the next 15 months on prednisone (0.4 mg/kg PO q 48 h) and azathioprine (2 mg/kg daily). During this period, the dog developed immune-mediated hemolytic anemia and thrombocytopenia, decubital ulcers, nasal aspergillosis, and eventually, multi-organ septicemia, which led to euthanasia on day 784. A diagnosis of pure white cell aplasia was made in this dog, based on the many similarities to human patients with pure white cell aplasia, including severe neutropenia with selective granulocyte aplasia, serum antineutrophil antibodies, remission dependent on treatment with immunosuppressive therapy, and recurrent bacterial infections.
一只3岁的爱尔兰猎狼犬因突然出现嗜睡和发热而接受评估。严重中性粒细胞减少（0/微升；参考区间为2500 - 11200/微升）与骨髓粒细胞发育不全相关（髓系：红系比例为0.009:1）。使用流式细胞术通过间接免疫荧光测定法评估抗中性粒细胞抗体。当正常犬类白细胞与患者血清及抗IgG一起孵育时，观察到中性粒细胞群体的前向角光散射有明显变化，单核细胞簇消失，这可能是破碎或溶解的结果。患者血清中中性粒细胞荧光强度（309 ± 11中位数通道单位[MCU]，对照值为107 - 152 MCU）以及荧光强度增加的中性粒细胞百分比（61 ± 5%，对照值为3.8 - 13.7%）均升高，这与抗中性粒细胞抗体的存在一致。在开始并调整使用甾体和非甾体免疫抑制疗法治疗期间，中性粒细胞减少反复发生。中性粒细胞计数最终稳定在低正常范围内，该犬在接下来的15个月中持续使用泼尼松（0.4毫克/千克口服，每48小时一次）和硫唑嘌呤（2毫克/千克/天）。在此期间，该犬患上了免疫介导的溶血性贫血和血小板减少症、褥疮性溃疡、鼻曲霉病，最终发展为多器官败血症，导致在第784天实施安乐死。基于与人类纯白细胞发育不全患者的诸多相似之处，包括严重中性粒细胞减少伴选择性粒细胞发育不全、血清抗中性粒细胞抗体、缓解依赖免疫抑制疗法治疗以及反复细菌感染，对该犬做出了纯白细胞发育不全的诊断。
