Systemic capillary leak syndrome.
Systemic capillary leak syndrome is a rare, severe disorder with a high mortality rate. It consists of the shift of fluid and proteins from the intravascular to the extravascular compartment with subsequent hypovolemic shock. We describe a 34-year-old-woman who had several episodes of generalized edema that evolved to hypovolemic shock. During the acute phase, laboratory investigations revealed marked hypoproteinemia, leukocytosis, and high levels of hematocrit and hemoglobin. A paraprotein IgG kappa chain was detected. Although different therapeutic trials were used, the patient continued to have similar episodes and she died during an acute episode 2 1/2 years after the first symptom of this disorder. The cause of systemic capillary leak syndrome is unknown. The presence of a paraprotein IgG is frequent in this group of patients.
Barnadas
M A
MA
Department of Dermatology, Hospital de la Sta. Creu i St. Pau, Barcelona, Spain.
Cisteró
A
A
Sitjas
D
D
Pascual
E
E
Puig
X
X
de Moragas
J M
JM
D000328:Adult; D002199:Capillary Permeability; D004487:Edema / Q000209:etiology*; D017809:Fatal Outcome; D005260:Female; D006400:Hematocrit; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D007019:Hypoproteinemia / Q000209:etiology; D007022:Hypotension / Q000209:etiology; D007074:Immunoglobulin G / Q000032:analysis; D007145:Immunoglobulin kappa-Chains / Q000032:analysis; D007964:Leukocytosis / Q000209:etiology; D010266:Paraproteins / Q000032:analysis; D012769:Shock / Q000209:etiology*; D013577:Syndrome
D006454:Hemoglobins; D007074:Immunoglobulin G; D007145:Immunoglobulin kappa-Chains; D010266:Paraproteins