Gaines J J, Tom G D, Khankhanian N
Department of Pathology, Medical College of Georgia, Augusta 30912-0300.
Hum Pathol. 1987 Nov;18(11):1160-4. doi: 10.1016/s0046-8177(87)80385-4.
The synovium in two well-documented cases of alkaptonuric ochronosis was studied by transmission electron and light microscopy. A feature of alkaptonuria previously unreported in the English-language literature was the presence of phagocytosis of large collagen fibrils by synovial macrophages in both cases. The origin of these fibrils appeared to have been shards of ochronotic cartilage and areas of metaplastic cartilage. This finding suggests that active remodeling of the synovial tissues occurs in advanced ochronotic arthropathy. Numerous shards of ochronotic cartilage were embedded in the synovium. In addition, small aggregates of large collagen fibrils encrusted with apparent ochronotic pigment were occasionally noted in the interstitium. These aggregates of ochronotic collagen are best described as microshards, and they have not generally been recognized in the literature. What appeared by light microscopy to represent ochronotic pigment deposition in interstitial collagen actually represented embedded microshards of ochronotic cartilage in the interstitium. Slender and elongated microshards were most likely to be confused by light microscopy as ochronotic interstitial collagen.
通过透射电子显微镜和光学显微镜对两例有充分记录的黑尿病性褐黄病患者的滑膜进行了研究。英文文献中此前未报道过的黑尿病的一个特征是,在这两例患者中滑膜巨噬细胞均出现了对大胶原纤维的吞噬现象。这些纤维的来源似乎是褐黄病性软骨碎片和化生软骨区域。这一发现表明,在晚期褐黄病性关节病中滑膜组织会发生活跃的重塑。大量褐黄病性软骨碎片嵌入滑膜。此外,在间质中偶尔会发现小的大胶原纤维聚集体,表面附着有明显的褐黄病色素。这些褐黄病胶原聚集体最好描述为微碎片,在文献中一般未被认识到。光学显微镜下看似间质胶原中褐黄病色素沉积的实际上是间质中嵌入的褐黄病性软骨微碎片。细长的微碎片在光学显微镜下最容易被误认为是褐黄病性间质胶原。