Abstract:
Capillary leak syndrome (CLS) is an increasingly acknowledged multifaceted and potentially lethal disease. Initial nonspecific symptoms are followed by the intriguing CLS hallmark: the double paradox associating diffuse severe edema and hypovolemia, along with hemoconcentration and hypoalbuminemia. Spontaneous resolutive phase is often associated with poor outcome due to iatrogenic fluid overload during leak phase. CLS is mainly triggered by drugs (anti-tumoral therapies), malignancy, infections (mostly viruses) and inflammatory diseases. Its idiopathic form is named after its eponymous finder: Clarkson\'s disease. CLS pathophysiology involves a severe, transient and multifactorial endothelial disruption which mechanisms are still unclear. Empirical and based-on-experience treatment implies symptomatic care during the acute phase (with the eventual addition of drugs amplifying cAMP levels in the severest cases), and the prophylactic use of monthly polyvalent immunoglobulins to prevent relapses. As CLS literature is scattered, we aimed to collect and summarize the current knowledge on CLS to facilitate its diagnosis, understanding and management.
摘要:
毛细血管渗漏综合征(CLS)是一种日益公认的多方面且可能致命的疾病。最初的非特异性症状之后是有趣的CLS标志:双重悖论与弥漫性严重水肿和低血容量有关,伴随着血液浓缩和低蛋白血症。由于在泄漏阶段医源性液体超负荷,自发的解决阶段通常与不良结果相关。CLS主要由药物(抗肿瘤疗法)引发,恶性肿瘤,感染(主要是病毒)和炎性疾病。它的特发性形式以其同名的发现者命名:克拉克森病。CLS病理生理学涉及严重的,短暂性和多因素内皮破坏的机制尚不清楚。经验性和基于经验的治疗意味着在急性期进行对症护理(在最严重的病例中,最终添加可增强cAMP水平的药物),以及每月预防性使用多价免疫球蛋白来预防复发。由于CLS文献分散,我们旨在收集和总结CLS的现有知识,以促进其诊断,理解和管理。