Complement consumption in a patient with necrobiotic xanthogranuloma and paraproteinemia.
Necrobiotic xanthogranuloma and paraproteinemia have been reported to be associated with acquired deficiency of the early components of the classic pathway of complement. We describe a patient in whom activation of complement was suggested by a high level of C3d, an accelerated catabolism of C1q, and a rapid consumption of C4 after an intravenous infusion of fresh-frozen plasma. This infusion was followed by an episode of pulmonary edema. Patients with complement deficiency or depletion should be monitored closely while receiving plasma, to avoid this potential complication.
Hauser
C
C
Department of Dermatology, Hôpital Cantonal Universitaire, Geneva, Switzerland.
Schifferli
J
J
Saurat
J H
JH
D015922:Complement C1q / Q000032:analysis; D003181:Complement C4 / Q000032:analysis; D003165:Complement System Proteins / Q000378:metabolism*; D006099:Granuloma / Q000097:blood* / Q000473:pathology; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007147:Immunoglobulin Light Chains / Q000032:analysis; D007145:Immunoglobulin kappa-Chains / Q000032:analysis; D008297:Male; D008875:Middle Aged; D009336:Necrosis; D010265:Paraproteinemias / Q000097:blood*; D012871:Skin Diseases / Q000097:blood* / Q000473:pathology; D014973:Xanthomatosis / Q000097:blood* / Q000473:pathology
D003181:Complement C4; D007074:Immunoglobulin G; D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D015922:Complement C1q; D003165:Complement System Proteins