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肿瘤溶解综合征:定义、发病机制、临床表现、病因及危险因素

Authors
Richard A Larson, MD
Ching-Hon Pui, MD
Section Editors
Reed E Drews, MD
Arnold S Freedman, MD
David G Poplack, MD
Deputy Editor
Diane MF Savarese, MD
Translators
曾铭, 主任医师,教授

引言

肿瘤溶解综合征(tumor lysis syndrome, TLS)是由大量肿瘤细胞溶解并释放大量的钾、磷和核酸进入体循环所造成的一种肿瘤急症。核酸分解代谢产生尿酸,导致高尿酸血症,尿酸排泄显著增加可导致尿酸沉积于肾小管,也可引起肾血管收缩、肾脏自动调节功能损害、肾血流量减少以及炎症,从而造成急性肾损伤。高磷血症及肾小管中的磷酸钙沉积也可引起急性肾损伤。

TLS最常发生在对高级别淋巴瘤(尤其是伯基特亚型)和急性淋巴细胞白血病(acute lymphoblastic leukemia, ALL)患者开始使用细胞毒治疗后。然而,TLS可自行发生,以及可在其他类型肿瘤存在增殖率较高、肿瘤负荷较大或对细胞毒治疗高度敏感时发生。

本文将总结TLS的发病机制、定义、分类、危险因素、病因、临床表现。关于TLS的预防和治疗将在别处讨论,与TLS相关的特定恶性肿瘤治疗相关问题也将单独讨论。 (参见“肿瘤溶解综合征的预防和治疗”“成人伯基特白血病/淋巴瘤的治疗”“成人T细胞白血病-淋巴瘤的治疗和预后”“儿童和青少年急性淋巴细胞白血病治疗的概述”,关于‘肿瘤溶解综合征’一节“急性髓系白血病并发症的概述”,关于‘肿瘤溶解综合征’一节)

发病机制

对增殖率高、肿瘤负荷大和/或对治疗高度敏感的恶性肿瘤开始使用细胞毒化疗、溶细胞抗体治疗或放疗可导致肿瘤细胞快速溶解,有时仅使用糖皮质激素治疗也可导致这种情况发生。 (参见下文‘病因及危险因素’)

这会使大量的细胞内物质(钾、磷和可代谢产生尿酸的核酸)释放进入体循环。所引起的代谢后果包括:高钾血症、高磷血症、继发性低钙血症、高尿酸血症以及急性肾损伤。同时出现的高水平的尿酸和磷酸盐会增加急性肾损伤的严重程度,因为在有磷酸钙结晶的情况下,尿酸易于沉积,而在有尿酸结晶的情况下,磷酸钙也易于沉积。

                

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Literature review current through: 2017-06 . | This topic last updated: 2016-09-09.
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