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Updated: May 10, 2026

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淋巴缺陷:分类,发病和治疗策略

Chaonan Zhang1, Jing Liu2, Yao Wang2

  • 1School of Clinical Medicine, Shandong Second Medical University, Weifang, Shandong, P.R. China; Laboratory of Translational Medicine in Microvascular Regulation, Medical Research Center, The First Affiliated Hospital of Shandong First Medical University & Shandong Provincial Qianfoshan Hospital, Jinan, Shandong, P.R. China; Shandong Provincial Key Medical and Health Laboratory of Translational Medicine in Microvascular Aging, Jinan, Shandong, P.R. China; Laboratory of Future Industry of Gene Editing in Vascular Endothelial Cells of Universities in Shandong Province, Jinan, Shandong, P.R. China.

Annals of vascular surgery
|May 25, 2025
PubMed
概括

淋巴发育不良 (LM) 是由影响PI3K/AKT/mTOR和VEGF等关键信号通路的遗传突变驱动的淋巴系统瘤. 目前的治疗包括mTOR抑制剂和VEGFR-3抑制剂,为患者提供了新的希望.

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Lymphatic vessels, known as lymphatics, are crucial in transporting lymph from peripheral tissues to our venous system. This process begins with lymph entering through tiny capillaries that branch through tissues. These capillaries have unique features such as larger diameters, thinner walls, and a distinctive one-way valve system formed by overlapping endothelial cells.
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The development of lymphatic tissues and vessels in embryonic life begins around the fifth week. These structures originate from the mesoderm layer, with lymph sacs emerging from developing veins.
The first lymph sacs to form are the paired jugular lymph sacs located at the junction of the internal jugular and subclavian veins. From these sacs, lymphatic capillary plexuses extend to the thorax, upper limbs, neck, and head, eventually forming lymphatic vessels. Each jugular lymph sac maintains a...