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Lymphomatoid granulomatosis in a 13-month-old infant
T J Lehman1, J A Church, H Isaacs
1Division of Pediatric Rheumatology, Hospital for Special Surgery, New York, NY 10021.
Insights
This study reports a rare case of lymphomatoid granulomatosis in a 13-month-old infant, highlighting the importance of considering this condition in pediatric failure to thrive cases.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Rare Diseases
Background:
- Lymphomatoid granulomatosis (LG) is a rare, progressive vasculitis.
- Typically affects middle-aged males, rarely diagnosed in early childhood.
Observation:
- A 13-month-old infant presented with chronic otitis media and failure to thrive.
- The infant exhibited hypogammaglobulinemia and persistent Epstein-Barr virus infection, suggesting immunocompromise.
Findings:
- This case represents one of the youngest reported instances of lymphomatoid granulomatosis.
- The infant's presentation underscores the need for broader differential diagnoses in pediatric failure to thrive.
Implications:
- Early recognition of lymphomatoid granulomatosis in infants is crucial for timely intervention.
- Further research is needed to understand the role of immunocompromise in pediatric lymphomatoid granulomatosis.
Abstract:
Lymphomatoid granulomatosis is an infrequent, progressive, and frequently fatal vasculitis which typically occurs in middle aged males. We describe a 13-month-old infant with lymphomatoid granulomatosis who presented with chronic otitis media and "failure to thrive." Although it is well recognized that rheumatic conditions may result in failure to thrive, pediatricians often fail to consider this possibility and the proper diagnosis and treatment are correspondingly delayed. Lymphomatoid granulomatosis occurs with increased frequency in immunocompromised patients. In our case both hypogammaglobulinemia and persistent evidence of Epstein-Barr virus infection suggest that the infant was immunocompromised. Whether these factors predisposed this infant to lymphomatoid granulomatosis is uncertain. No patient with the onset of lymphomatoid granulomatosis prior to 7 years of age has been reported.