Idiotype-specific intravenous immunoglobulin for therapy of immunoglobulin kappa free light chain deficiency

Hum Vaccin Immunother. 2019;15(5):1123-1125. doi: 10.1080/21645515.2019.1572411. Epub 2019 Mar 19.

Abstract

Deficient antibody production in patients with common variable immunodeficiency (CVID) is accompanied by an inability to produce free light chains (FLCs), particularly kappa (κ) FLC, due to B-cell dysfunction. We found that intravenous immunoglobulin (IVIg) administration, in a patient with CVID and (κ) FLC deficiency, for o short period of only 6 months, induced after discontinuation of treatment some kind of "long-lasting active immunity", leading to the secretion of immunoglobulin (κ) FLCs. A remarkable finding of our study is how effectively IVIg therapy led to a calculable (κ/λ) FLCs ratio, within the reference range. IVIg therapy may have functioned as an idiotype vaccine which induced a humoral response. To date, several questions remain open. For instance, from a clinical standpoint, we do not know whether this form of active immunotherapy has the potential to cure or just to control the immunoglobulin (κ) FLC deficiency. Further studies are necessary to confirm these findings.

Keywords: Intravenous immunoglobulin; common variable immunodeficiency; kappa free light chain deficiency; vaccine.

Publication types

  • Case Reports

MeSH terms

  • Administration, Intravenous
  • Adult
  • Common Variable Immunodeficiency / therapy*
  • Female
  • Humans
  • Immunoglobulin Light Chains
  • Immunoglobulins / deficiency*
  • Immunoglobulins / immunology
  • Immunoglobulins, Intravenous / therapeutic use*
  • Immunologic Deficiency Syndromes / diagnosis
  • Immunologic Deficiency Syndromes / immunology*
  • Immunologic Deficiency Syndromes / therapy*
  • Treatment Outcome

Substances

  • Immunoglobulin Light Chains
  • Immunoglobulins
  • Immunoglobulins, Intravenous

Supplementary concepts

  • Kappa-Chain Deficiency

Grants and funding

This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.