Inherited Deficiencies of the Late-Acting Complement Components Other than C9 Found among Healthy Blood Donors

Abstract

Among sera from 145,640 healthy blood donors in Osaka, 16 were found to have abnormalities in late-acting complement components other than C9. It was found that of these 16 sera, 2 were deficient in C5, 4 in C6, 6 in C7 and 4 in the C8 α-γ-subunit. The incidence of deficiency of each component among the Osaka blood donors was calculated as follows: C5 deficiency, 0.0014%; C6 deficiency, 0.0027%; C7 deficiency, 0.0041 %; C8 α-γ-subunit deficiency, 0.0027%. We confirmed that 13 donors were healthy and 12 had no past history related to a complement component deficiency. From these results, not only C9 deficiency but also deficiencies of the other late-acting complement components were found among the healthy blood donors, but no early-acting component deficiencies were noted.

High-Incidence of C9 Deficiency throughout Japan: There Are No Significant Differences in Incidence among Eight Areas of Japan

“…By further examination of these 145,640 blood donors, 138 were classified C9-deficient [1], and 16 were found to be deficient in late-act ing components other than C9 [2]. The incidence of C9 deficiency among blood donors in Osaka was de termined to be 0.095% [1].…”

Prevalence of hereditary properdin, C7 and C8 deficiencies in patients with meningococcal infections

“…They were not related and came from No C deficiency was found among 200 healthy volunteers of Sephardic and Ashkenazi origins who had no history of meningococcal disease. The C deficiency in the normal population was found in other studies to be quite rare (Ross & Densen, 1984;Inai et al, 1989).…”

“…C7 and C8 deficiencies appear to be associated in Moroccan and Yemenite Jews with susceptibility to meningococcal infection (Zimran et al, 1987, and our results). C9 deficiency is most common in Japan (Inaba et al, 1987;Inai et al, 1989) and is imposing increased risk of meningococcal meningitis on this population (Nagata et al, 1989). C6 deficiency is associated with meningococcal disease in Coloured and Black Africans in Cape Town, South Africa (Orren et al, 1987).…”

Terminal Complement Component Deficiencies in Japan

“…In order to determine the frequency of deficiency for each component of the complement system, a large-scale screening was performed [4,5]. As a first step, a hemolytic assay …”

“…By further examinations including hemolytic and protein assays of each of the components, component-deficient sera could be distinguished from sera with low CH50 activity caused by nonspecific activation of complement. Among 145,640 donors in the Osaka area (Japan), we found 2 cases with inherited C5D, 4 with C6D, 6 with C7D, 4 with C8 ·-Á subunit deficiency (C8·-ÁD) and 138 with C9D with an indcidence of 0.0014, 0.0027, 0.0041, 0.0027 and 0.0948%, respectively [4,5]. Hayama et al [6] reported that among 92,686 sera from hospitalized patients throughout Japan, 80 were found to be deficient in C9 using the same screening system.…”