Six Case Reports Of Systemic Capillary Leakage Syndrome In Adult

Systemic capillary leakage syndrome is a rare entity characterized by recurrent and unpredictable attacks of capillary leakage of plasma fluid and proteins throughout the endothelium.Since 1960 Clarkson et al first described a young woman presenting with recurrent episodes of shock due to "sudden phenomenal loss of plasma from her vascular bed there were only 200-300 similar cases of what was later termed the"systemic capillary leakage syndrome(SCLS)".Most of the documented subjects are adult,who experienced with severe hypotension,hypoalbuminemia,hemoconcentration,and generalized edema.It can be idiopathic or secondary,the former is known as Clarkson disease,causes of secondary SCLS include sepsis,malignant hematological diseases,such as lymphoma,myeloproliferative disorder;infectious disease,for example,hemorrhagic fever and medications which consists of IL-1,IL-11,gemcitabine and so on.Acute pancreatitis,trauma,post abdominal surgery were also reported to be the pathogens.Here,we described six cases of SCLS,the first three subjects were idiopathic SCLS,the last three were considered to be secondary SCLS.From this 6 cases,we hope to have a deep insight into the demographic character,nature clinical feature and the therapy efficacy of this disease which can also provide clinical data for timely diagnosis and medications.