ULTRASTRUCTURE AND 3-DIMENSIONAL ORGANIZATION OF THE TELANGIECTASES OF HEREDITARY HEMORRHAGIC TELANGIECTASIA
ULTRASTRUCTURE AND 3-DIMENSIONAL ORGANIZATION OF THE TELANGIECTASES OF HEREDITARY HEMORRHAGIC TELANGIECTASIA
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DOI：
10.1111/1523-1747.ep12555569
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发表时间：
1990-10-01
影响因子：
6.5
通讯作者：
JACOBSON, BS
中科院分区：
文献类型：
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作者：
BRAVERMAN, IM;KEH, A;JACOBSON, BS
对10例遗传性出血性毛细血管扩张症(HHT)的皮肤毛细血管扩张性病变进行了光镜和电子显微镜观察。用计算机对4个有代表性的病灶进行1 mm或2 mm塑料包埋切片重建。临床上最早可检测到的病变是毛细血管后小静脉的局灶性扩张，这些小静脉继续扩大，最终通过毛细血管与扩张的小动脉相连。随着血管病变的扩大，毛细血管段消失，形成直接的动-静脉交通。这一系列的形态学事件与血管周围单个核细胞的浸润有关，其中大多数细胞是淋巴细胞，少数是超微结构的单核/巨噬细胞。将这些发现与硬皮病和樱桃状血管瘤的毛细血管扩张垫相比较，发现前者由扩张的毛细血管后小静脉组成，也与血管周围的浸润物有关，而后者由毛细血管环动脉瘤产生，与之无关。
We studied 10 cutaneous telangiectatic lesions of hereditary hemorrhagic telangiectasia (HHT), ranging in size from pinpoint to 2 mm, by light and electron microscopy. Four representative lesions were reconstructed by computer from serial 1- or 2-mm plastic embedded sections. The earliest clinically detectable lesion of HHT is a focal dilatation of postcapillary venules, which continue to enlarge and eventually connect with dilated arterioles through capillaries. As the vascular lesion increases in size, the capillary segments disappear and a direct arterio-venous communication is formed. This entire sequence of morphologic events is associated with a perivascular mononuclear cell infiltrate in which the majority of cells are lymphocytes and the minority are monocytes/macrophages by ultrastructure. Comparison of these findings with the telangiectatic mats of scleroderma and cherry angiomas revealed that the former, previously shown to be composed of dilated postcapillary venules, are also associated with perivascular infiltrates, but the latter, which are produced by capillary loop aneurysms, are not.