Kaiserling E, Kröber S
Institute of Pathology, University of Tübingen, Germany.
Histopathology. 1994 Mar;24(3):215-21. doi: 10.1111/j.1365-2559.1994.tb00513.x.
Although amyloid deposition in relation to blood vessels is a well-recognized feature of generalized amyloidosis, lymphatic vessel amyloidosis is not mentioned in the literature. Systematic investigation of tissue removed at autopsy from patients with generalized amyloidosis and biopsy specimens from cases of localized amyloidosis and familial Mediterranean fever showed that amyloid deposition around lymphatics is by no means uncommon. The material investigated was mainly large and small bowel, lung, heart and kidney. Amyloid was identified by green birefringence with the Congo red stain on cross-polarization and lymphatics by their lack of immunostaining for CD34. Involvement of lymphatics was noted in 20 of the 42 organs from which specimens were examined, and was always accompanied by involvement of blood vessels and/or the interstitium. In the intestine, lymphatic amyloidosis was found mainly in the submucosa and subserosa, and was also demonstrated by electronmicroscopy in one case. Although lymphatic amyloidosis was equally common in the heart, lung and kidney, it was usually less prominent here than in the intestine. No lymphatic involvement was seen in localized amyloidosis. As the lymphatics play a central role in the resorption of interstitial proteins, they are probably also involved in the resorption of amyloid proteins. Amyloid deposition in the vicinity of lymphatics is probably the result of decompensation of this process.
尽管血管相关的淀粉样蛋白沉积是全身性淀粉样变性的一个公认特征，但淋巴管淀粉样变性在文献中并未被提及。对全身性淀粉样变性患者尸检切除的组织以及局限性淀粉样变性和家族性地中海热病例的活检标本进行系统研究发现，淋巴管周围的淀粉样蛋白沉积绝非罕见。所研究的材料主要是大小肠、肺、心脏和肾脏。通过刚果红染色在交叉偏振下呈现绿色双折射来鉴定淀粉样蛋白，通过对CD34缺乏免疫染色来鉴定淋巴管。在检查标本的42个器官中的20个中发现了淋巴管受累，并且总是伴有血管和/或间质受累。在肠道中，淋巴管淀粉样变性主要见于黏膜下层和浆膜下层，在1例中通过电子显微镜也得到了证实。尽管淋巴管淀粉样变性在心脏、肺和肾脏中同样常见，但在这里通常不如在肠道中明显。在局限性淀粉样变性中未见淋巴管受累。由于淋巴管在间质蛋白的重吸收中起核心作用，它们可能也参与了淀粉样蛋白的重吸收。淋巴管附近的淀粉样蛋白沉积可能是这个过程失代偿的结果。
