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: At a referral hospital for infectious diseases in Teresina, northeastern Brazil, medical records from deceased inpatients and survivors with VL from 1996 to 2005 were randomly selected. Only patients with typical symptoms plus a positive direct bone marrow (BM) smear, BM culture or indirectimmunofluorescence test (Biomanguinhos, Rio de Janeiro, Brazil) at dilution = 1:80 were included.