22q11.2 deletion syndrome with life-threatening adenovirus infection

Area of Science:

• Immunology
• Virology
• Pediatrics

Background:

• Adenovirus infections pose a severe threat to immunocompromised children, often leading to significant illness and death.
• Complete DiGeorge syndrome (22q11.2 deletion) severely impairs T-cell immunity, increasing susceptibility to opportunistic infections like adenovirus.

Observation:

• A pediatric patient with complete DiGeorge syndrome presented with life-threatening, high-level adenoviremia.
• Standard treatments were insufficient to control the aggressive viral load.

Findings:

• An infusion of T lymphocytes from a Human Leukocyte Antigen (HLA)-matched sibling donor was administered.
• Rapid and complete eradication of adenoviremia was observed following the T-cell infusion.
• The patient successfully reconstituted a diverse, donor-derived, postthymic T-cell repertoire, indicating immune recovery.

Implications:

• T-cell therapy offers a promising strategy for managing severe adenovirus infections in immunocompromised children.
• This approach may be particularly effective in patients with primary immunodeficiencies like DiGeorge syndrome.
• Successful immune reconstitution following T-cell therapy highlights its potential for long-term recovery and protection against infections.