Font R L, Rao N A, Issarescu S, McEntee W J
Arch Ophthalmol. 1978 Aug;96(8):1431-6. doi: 10.1001/archopht.1978.03910060179016.
A 52-year-old man had a prolonged history of nondeforming migratory polyarthritis and a short episode of pericarditis preceding the onset of bilateral vitreitis and retinitis. The clinical course was characterized by progressive deterioration of vision, increasing lethargy, and dementia, leading to coma and death from pneumonia (21 months later). No intestinal manifestations were recorded. Both eyes, which were removed postmortem, disclosed numerous PAS-positive macrophages throughout the inner retina and vitreous. Electron microscopic studies of the macrophages displayed intracytoplasmic, degenerating, rod-shaped bacteria and membranous structures identical to those seen in the intestine, brain, heart, and other tissues of patients with Whipple's disease. Clinicians should include Whipple's disease, and reticulum cell sarcoma, in the differential diagnosis of patients with bilateral retinitis and vitreitis, especially if these disorders are associated with CNS manifestations.
一名52岁男性,在双侧葡萄膜炎和视网膜炎发作之前,有长期的非变形性游走性多关节炎病史,以及一段短暂的心包炎病史。临床病程的特点是视力逐渐恶化、嗜睡加重和痴呆,最终导致昏迷,并因肺炎死亡(21个月后)。未记录到肠道表现。死后摘除的双眼显示,整个视网膜内层和玻璃体中有大量PAS阳性巨噬细胞。对巨噬细胞的电子显微镜研究显示,胞浆内有退化的杆状细菌和膜性结构,与惠普尔病患者的肠道、大脑、心脏和其他组织中所见的结构相同。临床医生在对双侧视网膜炎和葡萄膜炎患者进行鉴别诊断时,应考虑惠普尔病和网状细胞肉瘤,特别是当这些疾病伴有中枢神经系统表现时。