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Complement activation in patients with immune thrombocytopenic purpura according to phases of disease course

2020/06/09 by Roberto Castelli, R Castelli, G Lambertenghi Delilliers +6
Immunology and Microbiology · Medicine · #Blood groups and transfusion #Complement system in diseases #Platelet Disorders and Treatments

paper · doi:10.1111/cei.13475

openalex publication_date 2020/06/09 · crossref created 2020/06/09 · crossref issued 2020/07/06 · crossref published 2020/07/06 · crossref published-online 2020/07/06 · crossref published-print 2020/08/12 · crossref deposited 2022/01/01 · openalex created_date 2025/10/10 · openalex updated_date 2026/07/28 · crossref indexed 2026/07/28

Abstract

Immune thrombocytopenic purpura (ITP) is an autoimmune thrombocytopenia with shortened platelet survival and relative bone marrow failure. The pathogenesis involves antibody production, cytokine release, T cell impairment, complement activation and clearance of platelets. We measured plasma levels of C3, C4, C1q and sC5b-9 in 80 ITP patients in acute phase, 50 ITP patients in complete (CR) or partial (PR) remission and 50 age- and sex-matched healthy volunteers. Statistical analyses showed that acute ITP patients had higher plasma levels of sC5b-9 and C1q than CR or PR patients (median = sC5b-9: 200 versus 98 mg/dl, P-value < 0·001) (median C1q = 2·11 versus 1·00 mg/dl, P-value < 0·001). CR and PR ITP patients had sC5b-9 and C1q plasma levels comparable to those observed in healthy volunteers. There was a significant correlation between sC5b-9 and C1q plasma levels (Spearman's rho correlation index on 130 ITP patients equal to 0·58, P-value < 0·001). We also found that sC5b-9 plasma level is inversely correlated with the number of platelets. Furthermore, we divided acute ITP patients into subjects with detectable (24 of 80, 30%) or undetectable (56 of 80, 70%) anti-platelet antibodies; patients with detectable anti-platelet antibodies have significantly higher plasma levels of C1q and sC5b-9. This research will potentially offer novel therapeutic strategies in light of new drugs affecting complement activation for monitoring therapy response.

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