The systemic capillary leak syndrome is a rare condition with episodic capillary hyperpermeability resulting in protein and fluid crossing into the interstitial space.


Clinical and laboratory features:

(1) diffuse swelling and edema

(2) weight gain

(3) renal failure

(4) elevated hematocrit

(5) hypoalbuminemia and hypoproteinemia

(6) hypovolemic shock associated with marked reduction in plasma volume

(7) no impairment of consciousness


Occasional findings:

(1) myalgias with or without rhabdomyolysis

(2) symptoms of nerve compression

(3) fatigue

(4) monoclonal protein in the serum in the absence of myeloma

(5) preceded by fever (in 30%)


Cases may be:

(1) idiopathic

(2) drug-induced (retinoids, all-trans retinoic acid, docetaxel)

(3) secondary to malignant lymphoma

(4) secondary to therapy with humanized anti-CD22 monoclonal antibodies

(5) secondary to erythrodermic psoriasis

(6) secondary to an upper respiratory tract infection


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