Paradoxical reconstitution of complement activity following plasma transfusion of an individual with deficiency of the seventh component of complement.
A subject deficient in the seventh component of complement (C7) was plasmapheresed with 660 ml C7-sufficient plasma. The expected reconstitution of C7 activity, followed by exponential decay, was not observed. During day 1, serum haemolytic C7 and total haemolytic complement were undetectable and C7 levels were very low by C7 ELISA. However, low levels of circulating fluid phase terminal complement complex (TCC) were detected. On day 2 about microgram C7/ml serum was detected and this rose to 6 micrograms/ml by day 17. Functional complement activity was also present. At day 28 the serum C7 and total haemolytic complement had dropped to pretransfusion levels. A low level of C5b6 was present in pretransfusion serum and this increased markedly immediately following transfusion when the patient's serum also acquired C7 consuming activity. Throughout the study low levels of anti-C7 antibodies were present but there was no evidence that antibody was directly responsible for the C7 consumption. Nevertheless antibody-antigen interactions could have generated circulating C5b6. C5b6 has been shown previously to have the capacity to inhibit C7 activity in vitro. Investigations of the C7 circulating on days 2-17 demonstrated normal molecular weight, functionally active C7. The donor sera and the recirculating C7 allotyped C7-1 by isoelectric focusing; however, the recirculating C7 showed additional weak bands with C7 functional activity, suggesting a possible genetic or acquired abnormality. Although the disappearance of C7 immediately post-transfusion may be explained by the presence of C5b6, there is no satisfactory explanation for the rising C7 levels on days 2-17 and we cannot exclude temporary C7 secretion by the patient.
Platonov
A E
AE
Central Institute of Epidemiology, Moscow, Russia.
Würzner
R
R
Beloborodov
B
B
Jones
A M
AM
Troshansky
D V
DV
Vershinina
I V
IV
Lachmann
P J
PJ
Orren
A
A
D000328:Adult; D002908:Chronic Disease; D003184:Complement C7 / Q000032:analysis / Q000172:deficiency*; D015941:Complement Hemolytic Activity Assay; D015938:Complement Membrane Attack Complex / Q000378:metabolism; D003165:Complement System Proteins / Q000378:metabolism; D006801:Humans; D008297:Male; D010035:Otitis Media, Suppurative / Q000276:immunology* / Q000628:therapy; D010956:Plasmapheresis
D003184:Complement C7; D015938:Complement Membrane Attack Complex; C050974:complement C5b-6 complex; D003165:Complement System Proteins