Immune complexes and complement profile in essential mixed cryoglobulinemia before and after plasma exchange.
Five patients affected by essential mixed cryoglobulinemia (EMC) with renal involvement unresponsive to high doses of corticosteroids, have been treated with 16 plasma exchanges (PE). The plasma removed at each apheresis was 1652 +/- 416 ml. The following data were evaluated before and after PE: levels of immune complexes as detected by cryocrit and C1q binding assay; total complement activity (CH50) and alternative pathway complement activity (APCH 50); concentrations of 9 complement components (C1q, C1s, C4, C3, C5, C6, B, I and H) and of the C3 split product C3d. By definition all serum samples had detectable cryoprecipitates and 4 out 5 had C1q binding activity higher than the upper limit of 2 SD. The effect of the PE was the decrease of cryocrit and of C1q binding activity approximately to one half their initial values. The basal complement profile suggested and excessive activation of complement through the classical pathway. Indeed we found low levels of the early complement components (C1q, C1s and C4), reduced CH50, normal levels of C3 and an increase of C3 split product C3d concentrations. APCH50 as well as C5, C6, B, I and H concentrations were found within the normal range. After each PE a significant decrease of the previously normal components was observed. The decrease was independent of the replacement fluid used (5% albumin solution or fresh plasma or cryoglobulin-depleted autologous plasma) and had a short duration. Indeed all these components after the drop following the PE increased rapidly reaching the basal level over a period of 24-48 hours.(ABSTRACT TRUNCATED AT 250 WORDS)
D000936:Antigen-Antibody Complex / Q000032:analysis*; D003165:Complement System Proteins / Q000032:analysis* / Q000276:immunology; D003449:Cryoglobulinemia / Q000276:immunology* / Q000628:therapy; D006801:Humans; D010265:Paraproteinemias / Q000276:immunology*; D010951:Plasma Exchange
D000936:Antigen-Antibody Complex; D003165:Complement System Proteins