Disease pathophysiology: Systemic capillary leak syndrome is a rare disorder characterized by self-reversing episodes during which the endothelial cells which line the capillaries are thought to separate for a few days, allowing for massive leakage of plasma and other blood components from the blood vessels into neighbouring body cavities and muscles. Systemic capillary leak syndrome usually consists of two phases i.e capillary leak phase and recruitment phase. This leads to swelling. Systemic capillary leak syndrome leads to hypotension, hemoconcentration, and hypoalbuminemia. It is a life threatning syndrome often misdiagnosed with polycythemia,polycythemia vera, or sepsis.
Treatment: Mainly the intravenous administration of saline solution plus hetastarch or albumin and colloids (to increase the remaining blood flow to vital organs like the kidneys), as well as glucocorticoids (steroids like methylprednisolone, to reduce or stop the capillary leak). However, it is important to avoid overly aggressive intravenous fluid administration during this leak phase, because it may cause massive swelling of the extremities and thus serious collateral damage because of induced compartment syndromes.
The patient was diagnosed with SCLCS and treated with therbutaline and aminophylline. Research: Major research is been performed by NORD (National Organization of Rare Disorders) Statistics: Systemic capillary leak syndrome was first described by Clarkson in 1960 and a literature review in 2002 listed 57 case reports of patients presenting with this condition. Of these 57 patients, 82% had an associated mono-clonal immunoglobulin band on plasma electrophoresis; mainly IgG kappa, as occurred in this case.