Durand Bechu Manon, Rouget Antoine, Recher Christian, Azoulay Elie, Bounes Vincent
Service d'Aide Médicale Urgente de la Haute Garonne (SAMU 31), Hôpital Universitaire de Purpan, place du Docteur Baylac, TSA 40031, 31059 Toulouse Cedex 9, France.
Service d'Anesthésie-Réanimation, CHU Hôpital Rangueil, 1 avenue Jean Poulhes, TSA 50032, 31059 Toulouse Cedex 9, France.
Case Rep Emerg Med. 2016;2016:5347039. doi: 10.1155/2016/5347039. Epub 2016 Mar 16.
Systemic Capillary Leak Syndrome (SCLS) is a rare disease with poor prognosis, characterized by the occurrence of mucocutaneous and visceral edema with hypotension, hemoconcentration, and unexpected hypoalbuminemia. The disease can be idiopathic (Clarkson syndrome) or secondary to other diseases and treatments. We describe this syndrome in a prehospitalized, 63-year-old patient with chronic lymphocytic leukemia and an idiopathic form of SCLS manifesting as hypovolemic shock. Initial care is hospitalization in intensive care. In addition to etiological treatment if fluid replacement is necessary, treatment must be closely monitored for secondary overload complications. Catecholamine rather than arrhythmogenic support may be associated.
系统性毛细血管渗漏综合征（SCLS）是一种预后不良的罕见疾病，其特征为出现黏膜皮肤和内脏水肿，并伴有低血压、血液浓缩和意外的低白蛋白血症。该疾病可为特发性（克拉克森综合征）或继发于其他疾病及治疗。我们描述了一名63岁的慢性淋巴细胞白血病患者，其在住院前出现特发性SCLS，表现为低血容量性休克。初始治疗是入住重症监护病房。除了病因治疗（如有必要进行液体补充）外，必须密切监测治疗过程中的继发性容量超负荷并发症。可能需要使用儿茶酚胺而非抗心律失常药物进行支持治疗。
