Acute renal failure associated with systemic capillary leak syndrome

Systemic capillary leak syndrome (SCLS) is rare disorder characterized by unexplained episodic capillary hyperpermeability, resulting in generalized edema and hypovolemic shock. During acute episodes, there is characteristic elevated hematocrit and reduced serum protein. We report a 55-year-old man who had two recurrent episodes of SCLS. Laboratory findings revealed severe hypoalbuminemia, sudden elevation of hematocrit, and paraprotein IgG κ chain in his serum. The patient had acute oliguric renal failure during the acute episode. Despite receiving a large amount of fluid resuscitation, his hematocrit rose to 71.4%. After combination therapy of venesection and fluid resuscitation, massive diuresis occurred, and the symptoms were alleviated. We found that venesection was effective in controlling the acute episode of SCLS, without troublesome adverse effects. Although prophylactic therapy with terbutaline and theophylline is effective for SCLS, treatment in the acute phase is still empirical. We conclude that fluid replacement therapy accompanied by venesection is an alternative regimen in patients with severe capillary leakage.