Cell-free IgG-aggregates in plasma of patients with chronic lymphocytic leukemia cause chronic activation of the classical complement pathway

PLoS One. 2020 Mar 9;15(3):e0230033. doi: 10.1371/journal.pone.0230033. eCollection 2020.

Abstract

Therapy regimens for Chronic lymphocytic leukemia (CLL) commonly include chemotherapy and immunotherapy, which act through complement-mediated-cytotoxicity (CDC) and other mechanisms. CDC depends on several factors, including the availability and activity of the complement classical pathway (CP). Recently, a significant decrease in CP activity was shown to be associated with an immunoglobulin-C5a complex (Ig-C5a) and other markers of chronic CP activation in 40% of the patients. The study focused on the involvement of IgG-hexamers, an established CP activator, in the mechanism of chronic CP activation in CLL. Sera from 51 naïve CLL patients and 20 normal controls were collected. CP and alternative pathway (AP) activities were followed by the complement activity marker sC5b-9. Serum high molecular weight (HMW) proteins were collected by gel-filtration chromatography and their complement activation capacity was assessed. The levels of IgM, another established CP activator, were measured. Data were associated with the presence of Ig-C5a. Baseline levels of activation markers negatively correlated with CP and the AP activities, supporting chronic complement activation. In patients with Ig-C5a, HMW proteins that are not IgM, activated the complement. HMW proteins were identified as IgG-aggregates by affinity binding assays and Western blot analysis. The data indicate chronic CP activation, mediated by cell-free IgG-hexamers as a cause of decreased CP activity in part of the CLL population. This mechanism may affect immunotherapy outcomes due to compromised CP activity and CDC.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Complement Pathway, Classical*
  • Female
  • Humans
  • Immunoglobulin G / blood*
  • Immunoglobulin G / chemistry
  • Leukemia, Lymphocytic, Chronic, B-Cell / blood*
  • Leukemia, Lymphocytic, Chronic, B-Cell / immunology*
  • Male
  • Middle Aged
  • Molecular Weight

Substances

  • Immunoglobulin G

Grants and funding

This research was partly supported by: 1. The Dangoor foundation (granted via the Azrieli Faculty of Medicine, Bar Ilan University, Safed, Israel), grant number 292956, PI: A. Braester. 2. The Health Corporation of Galilee Medical Center (Nahariya, Israel), PI: A. Braester. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.