In Case of Severe Hemoconcentration and Hypovolemic Shock, Also Think of Zebras: A Case of Systemic Capillary Leak Syndrome

Systemic capillary leak syndrome (SCLS) is a rare disease characterized by recurrent episodes of hypotension, hemoconcentration, and edema due to capillary leakage of protein-rich fluids to the interstitial space. Fluid therapy is the mainstay of treatment; however, this may worsen symptoms leading to organ dysfunction and compartment syndrome in severe cases. This case report describes a patient who presented with tachycardia, hypotension, and hemoconcentration. Clinical assessment suggested hypovolemic shock and fluid therapy was initiated. Nevertheless, there was progressive clinical deterioration with increasing hemoconcentration and development of edema in all extremities, matching the SCLS diagnosis. Fluid therapy was stopped but compartment syndrome could not be prevented. Fasciotomy was performed, and treatment with imatinib, a selective tyrosine kinase inhibitor, and intravenous immunoglobulins (IVIG) was started. After 20 days, the patient was discharged home. Exact pathogenesis and disease-specific treatment for SCLS remain unclear. Based on pathophysiological grounds, both imatinib and IVIG may be effective in restoring the profound derangements of the vascular endothelium. Several case reports describe the success of IVIG in SCLS, and intermittent treatments may prevent future episodes. In addition, early diagnosis, frequent evaluation of applied therapy, and early recognition of acute compartment syndrome are important as they may prevent further damage.