特发性系统性毛细血管渗漏综合征(ISCLS)是一种罕见的疾病,由于血管内皮通透性增加,血管内液体和蛋白质外渗到间质空间。它的特点是低血压发作,低蛋白血症,和全身水肿的血液浓缩。其病因未知。然而,在80%以上的病例中,它与单克隆丙种球蛋白病有关。目前没有针对性的治疗,危机期间的方法是支持性的,主要是控制血压,维持重要器官的灌注,预防并发症,如急性肺水肿和因缺血引起的器官衰竭,这是死亡的主要原因。我们介绍了一个72岁的男性,患有全身性水肿和胸膜,心包,和腹膜积液,其实验室结果显示低蛋白血症,低蛋白血症,免疫球蛋白Gκ单克隆丙种球蛋白病。在一项详尽的补充研究后,排除了严重低白蛋白血症伴anasarca的其他病因。导致与单克隆丙种球蛋白病相关的ISCLS的诊断。患者在白蛋白和利尿剂治疗下表现出进行性临床改善。然而,他们因低血压合并多器官功能障碍而再次入院,几小时后死亡。
Idiopathic systemic capillary leak syndrome (ISCLS) is a rare condition caused by the extravasation of intravascular fluids and proteins into the interstitial space due to increased vascular endothelium permeability. It is characterized by episodes of hypotension, hypoalbuminemia, and hemoconcentration with generalized edema. Its etiopathogenesis is unknown. However, it is associated with monoclonal gammopathy in more than 80% of cases. There is currently no targeted treatment, and the approach during a crisis is supportive, mainly to control blood pressure, maintain perfusion of vital organs, and prevent complications, such as acute pulmonary edema and organ failure due to ischemia, which are the primary causes of death. We present the case of a 72-year-old man with generalized edema and pleural, pericardial, and peritoneal effusions whose laboratory results showed hypoalbuminemia, hypoproteinemia, and immunoglobulin G kappa monoclonal gammopathy. Other etiologies for severe hypoalbuminemia with anasarca were excluded after an exhaustive complementary study, leading to the diagnosis of ISCLS associated with monoclonal gammopathy. The patient showed progressive clinical improvement with albumin and diuretic therapy. However, they were readmitted to the hospital due to hypotension with multiorgan dysfunction and died a few hours later.