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Paradoxical reconstitution of complement activity following plasma transfusion of an individual with deficiency of the seventh component of complement.

机译:缺乏补体第七成分的个体血浆输注后补体活性的悖论性重构。

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摘要

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.
机译:用660 ml足够的C7血浆对补体(C7)第七成分不足的受试者进行血浆置换。没有观察到预期的C7活性重构,然后是指数衰减。在第1天,通过C7 ELISA检测不到血清溶血C7和总溶血补体,并且C7水平非常低。但是,检测到低水平的循环液相末端补体复合物(TCC)。在第2天,检测到约微克C7 / ml血清,到第17天,血清升至6微克/ ml。在第28天,血清C7和总溶血补体已降至输血前水平。输血前血清中存在低水平的C5b6,当患者的血清也具有消耗C7的活性时,输血后立即显着增加。在整个研究过程中,存在低水平的抗C7抗体,但没有证据表明抗体直接导致C7消耗。然而,抗体-抗原相互作用可能已经产生循环的C5b6。先前已证明C5b6具有体外抑制C7活性的能力。对第2-17天循环的C7的研究表明分子量正常,具有功能活性的C7。通过等电聚焦,将供体血清和循环C7异型为C7-1。然而,再循环的C7显示具有C7功能活性的另外的弱带,表明可能的遗传或获得性异常。尽管输血后立即出现的C7消失可以用C5b6的存在来解释,但是对于第2-17天C7水平的升高尚无令人满意的解释,我们不能排除患者暂时分泌C7的情况。

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