Tonsillar granuloma associated with hypogammaglobulinemia

Area of Science:

• Immunology
• Otorhinolaryngology

Background:

• Rare tonsillar granulomas can stem from infections, malignancies, or sarcoidosis.
• Inborn errors of immunity (IEI), like common variable immunodeficiency (CVID), can also manifest as granulomas, presenting diagnostic challenges due to overlapping features with sarcoidosis and drug-induced hypogammaglobulinemia.

Observation:

• A 29-year-old female with epilepsy presented with dysphagia and dyspnea due to swollen lingual tonsils and laryngeal edema.
• Biopsies revealed non-necrotizing granulomas, with no infectious etiology identified.
• Immunological evaluation showed hypogammaglobulinemia with impaired polysaccharide responses, consistent with CVID, potentially drug-induced.

Findings:

• The patient exhibited high CD21low B cells and low IgG levels, indicating a B cell defect.
• Symptoms of dysphagia and dyspnea improved with subcutaneous immunoglobulin (IgG) therapy and rituximab.

Implications:

• Tonsillar granulomas may be the primary and sole clinical manifestation of B cell deficiencies.
• This underscores the broad spectrum of clinical presentations in primary and secondary immunodeficiencies, necessitating comprehensive diagnostic workups.