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ECH是非郎格罕组织细胞增生症中的少见分型,由JakobErdheim和William Chester于1930年首先发现。据文献报道,截止到2010年11月止,仅发现不到400例。对于ECH的具体病理生理了解甚少。ECH的特征性组织学表现为多发的网织纤维细胞及富含脂质的巨噬细胞浸润。
ECH is a rare type of non-Langerhan histiocytosis, first discovered by Jakob Erdheim and William Chester in 1930. According to the literature, as of November 2010, only 400 cases were found. Little is known about the specific pathophysiology of ECH. The characteristic histological findings of ECH are multiple reticular fibroblasts and lipid-rich macrophage infiltration.