Severe Tick-Borne Encephalitis (TBE) in a Patient with X-Linked Agammaglobulinemia; Treatment with TBE Virus IgG Positive Plasma, Clinical Outcome and T Cell Responses

J Clin Immunol. 2024 Apr 27;44(5):116. doi: 10.1007/s10875-024-01718-5.

Abstract

Purpose: A patient with X-linked agammaglobulinemia (XLA) and severe tick-borne encephalitis (TBE) was treated with TBE virus (TBEV) IgG positive plasma. The patient's clinical response, humoral and cellular immune responses were characterized pre- and post-infection.

Methods: ELISA and neutralisation assays were performed on sera and TBEV PCR assay on sera and cerebrospinal fluid. T cell assays were conducted on peripheral blood the patient and five healthy vaccinated controls.

Results: The patient was admitted to the hospital with headache and fever. He was not vaccinated against TBE but receiving subcutaneous IgG-replacement therapy (IGRT). TBEV IgG antibodies were low-level positive (due to scIGRT), but the TBEV IgM and TBEV neutralisation tests were negative. During hospitalisation his clinical condition deteriorated (Glasgow coma scale 3/15) and he was treated in the ICU with corticosteroids and external ventricular drainage. He was then treated with plasma containing TBEV IgG without apparent side effects. His symptoms improved within a few days and the TBEV neutralisation test converted to positive. Robust CD8+ T cell responses were observed at three and 18-months post-infection, in the absence of B cells. This was confirmed by tetramers specific for TBEV.

Conclusion: TBEV IgG-positive plasma given to an XLA patient with TBE without evident adverse reactions may have contributed to a positive clinical outcome. Similar approaches could offer a promising foundation for researching therapeutic options for patients with humoral immunodeficiencies. Importantly, a robust CD8+ T cell response was observed after infection despite the lack of B cells and indicates that these patients can clear acute viral infections and could benefit from future vaccination programs.

Keywords: CD8+ T cells; X-linked agammaglobulinemia; immunodeficiency; neutralising antibodies; tetramers; tick-borne encephalitis.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Agammaglobulinemia* / immunology
  • Agammaglobulinemia* / therapy
  • Antibodies, Viral* / blood
  • Encephalitis Viruses, Tick-Borne* / immunology
  • Encephalitis, Tick-Borne* / diagnosis
  • Encephalitis, Tick-Borne* / immunology
  • Encephalitis, Tick-Borne* / therapy
  • Genetic Diseases, X-Linked* / immunology
  • Genetic Diseases, X-Linked* / therapy
  • Humans
  • Immunization, Passive / methods
  • Immunoglobulin G* / blood
  • Immunoglobulin G* / immunology
  • Male
  • T-Lymphocytes* / immunology
  • Treatment Outcome

Substances

  • Immunoglobulin G
  • Antibodies, Viral

Supplementary concepts

  • Bruton type agammaglobulinemia