Kawabe Shotetsu, Saeki Takako, Yamazaki Hajime, Nagai Masaaki, Aoyagi Ryuji, Miyamura Shoji
Department of Internal Medicine, Nagaoka Red Cross Hospital.
Intern Med. 2002 Mar;41(3):211-5. doi: 10.2169/internalmedicine.41.211.
A 40-year-old woman was referred to our hospital with severe hypovolemic shock and anasarca. The laboratory findings showed marked hemoconcentration and a decrease in total serum protein with the presence of monoclonal IgG-lambda. She had had a similar episode of generalized edema 2 years previously. We diagnosed the patient as having typical systemic capillary leak syndrome (SCLS) and she improved gradually after infusion of albumin-containing fluid. SCLS is a very rare condition caused by unexplained episodic capillary hyperpermeability. Its treatment has remained largely supportive and the prognosis is generally poor. Awareness of SCLS is necessary for improvement of the outcome.
一名40岁女性因严重低血容量性休克和全身水肿被转诊至我院。实验室检查结果显示明显的血液浓缩，血清总蛋白降低，伴有单克隆IgG-λ。她在两年前曾有过类似的全身性水肿发作。我们诊断该患者患有典型的系统性毛细血管渗漏综合征（SCLS），在输注含白蛋白的液体后病情逐渐好转。SCLS是一种由不明原因的间歇性毛细血管通透性增加引起的非常罕见的疾病。其治疗主要是支持性的，预后通常较差。提高对SCLS的认识对于改善治疗结果很有必要。
