Chronic idiopathic systemic capillary leak syndrome: a case report

Abstract

Background Idiopathic systemic capillary leak syndrome (ISCLS), is a rare disorder characterized by recurrent attacks of varying severity of hypovolemic shock and generalized edema in association with hemoconcentration and hypoalbuminemia in the absence of albuminuria. The chronic form of ISCLS is extremely rare with only a few cases reported in the literature. Case presentation Here we report the case of a young woman who developed chronic ISCLS characterized by massive, generalized and persistent edema. The work up confirmed the presence of monoclonal gammopathy. She was treated with several agents with no response. Conclusion Chronic ISCLS is a very rare disease of unknown etiology and results in devastating complications. The medical community should be aware of this disease with the hope that targeted therapy will become available in the future.

Chronic systemic capillary leak syndrome associated with an intravascular large B-cell lymphoma in a patient undergoing hemodialysis: a case report with literature review

“…Moreover, chronic SCLS induced by gemcitabine has also been reported [13,14]. Although there are no reports on SCLS in patients undergoing hemodialysis, Alkhunaizi et al described a case of idiopathic chronic SCLS in a patient who did not respond to several therapeutic agents and who developed acute kidney injury that required hemodialysis [11]. This patient had massive edema despite daily hemodialysis, which was suggestive of difficulty in body fluid removal during hemodialysis.…”

“…There are only a few case reports on the chronic form of SCLS [4,[10][11][12][13][14][15]. Unlike the classical form, patients with chronic SCLS experience mild progressive generalized edema, along with pleural and pericardial effusions without signs of spontaneous recovery.…”

Capillary hyperpermeability syndrome: A fatal complication of acute leukaemia: Case report and review

“…Clarkson's syndrome or capillary hyperpermeability syndrome was first reported in 1950 by Bayard Clackson and colleagues, hence its name. Since then, about 500 cases have been reported in the literature, mainly in young adults, but also 32 cases have been reported in children, such as ours [ 1 , 2 ].…”

Clinical features of 47 secondary hemophagocytic lymphohistiocytosis patients complicated with capillary leak syndrome

“…Currently, there are very few studies on sHLH-induced CLS, only a few case reports [ 12 – 14 ]. In clinical practice, we have found that HLH patients complicated with CLS had rapid disease progression and poor prognosis.…”