Kasten Philipp, Schnöink Gerrit, Bergmann Astrid, Papoutsi Maria, Buttler Kerstin, Rössler Jochen, Weich Herbert A, Wilting Jörg
Department of Anatomy and Cell Biology, Georg-August-University, Goettingen, Germany.
Dev Dyn. 2007 Oct;236(10):2952-61. doi: 10.1002/dvdy.21298.
Lymphangioma is a disfiguring malformation of early childhood. A mouse lymphangioma model has been established by injecting Freund's incomplete adjuvant (FIA) intraperitoneally, but has not been compared with the human disease. We show that, in accordance with studies from the 1960s, the mouse model represents an oil-granuloma, made up of CD45-positive leukocytes and invaded by blood and lymph vessels. Several markers of lymphatic endothelial cells are expressed in both mouse and human, like CD31, Prox1, podoplanin, and Lyve-1. However, the human disease affects all parts of the lymphovascular tree. We observed convolutes of lymphatic capillaries, irregularly formed collectors with signs of disintegration, and large lymph cysts. We observed VEGFR-2 and -3 expression in both blood vessels and lymphatics of the patients, whereas in mouse VEGFR-2 was confined to activated blood vessels. The experimental mouse FIA model represents a vascularized oil-granuloma rather than a lymphangioma and reflects the complexity of human lymphangioma only partially.
淋巴管瘤是一种影响儿童外貌的畸形病症。通过腹腔注射弗氏不完全佐剂(FIA)建立了小鼠淋巴管瘤模型,但尚未与人类疾病进行比较。我们发现,与20世纪60年代的研究一致,该小鼠模型表现为一种油肉芽肿,由CD45阳性白细胞组成,并被血管和淋巴管侵入。淋巴管内皮细胞的几种标志物在小鼠和人类中均有表达,如CD31、Prox1、足板蛋白和淋巴管内皮透明质酸受体1(Lyve-1)。然而,人类疾病会影响淋巴管系统的各个部位。我们观察到淋巴管毛细血管盘绕、不规则形成的集合管有解体迹象以及大的淋巴囊肿。我们在患者的血管和淋巴管中均观察到血管内皮生长因子受体2(VEGFR-2)和血管内皮生长因子受体3(VEGFR-3)的表达,而在小鼠中VEGFR-2仅限于活化的血管。实验性小鼠FIA模型代表的是一种血管化的油肉芽肿而非淋巴管瘤,仅部分反映了人类淋巴管瘤的复杂性。