Activation of the complement system in patients with porphyrias after irradiation in vivo.
Irradiation of the forearms of two patients with erythropoietic protoporphyria and one patient with porphyria cutanea tarda resulted in an in vivo activation of the complement system, as assessed by diminution of the hemolytic titers of the third component of complement by 23-57%, and of the fifth component of complement (C5) by 19-47%. Such treatment also generated chemotactic activity for human polymorphonuclear cells; the chemotactic activity was stable at 56 degrees C and antigenically related to human C5. On Sephadex G-75 chromatography the chemotactic activity eluted with an apparent molecular weight of 15,000. These in vivo results extend our previous in vitro observation of photoactivation of complement in sera from patients with erythropoietic protoporphyria and porphyria cutanea tarda, and suggest that the complement system may participate in the pathogenesis of cutaneous phototoxicity in these patients.
D002634:Chemotaxis, Leukocyte / Q000528:radiation effects; D003167:Complement Activation / Q000528:radiation effects*; D003176:Complement C3 / Q000032:analysis; D003182:Complement C5 / Q000032:analysis; D004920:Erythropoiesis; D006462:Hemolytic Plaque Technique; D006801:Humans; D008027:Light; D008297:Male; D008875:Middle Aged; D011164:Porphyrias / Q000276:immunology*; D011166:Porphyrins / Q000097:blood*; D011524:Protoporphyrins / Q000097:blood*; D012867:Skin / Q000528:radiation effects; D012871:Skin Diseases / Q000276:immunology*
D003176:Complement C3; D003182:Complement C5; D011166:Porphyrins; D011524:Protoporphyrins