Wiskott-Aldrich syndrome is a primary immunodeficiency syndrome in which the majority of malignant complications are non-Hodgkin's lymphoma. We report here a Wiskott-Aldrich syndrome patient who developed Epstein-Barr virus-positive Hodgkin's disease in the bilateral pulmonary hilar lymph nodes. The treatment was successful as the patient achieved a complete response and event-free survival for more than 4 y. Conclusion: This case is very rare, but highly suggestive of the immune-mediated mechanisms in the pathogenesis of Epstein-Barr virus-associated Hodgkin's disease in an immunodeficiency patient.
|Number of pages||4|
|Journal||Acta Paediatrica, International Journal of Paediatrics|
|Publication status||Published - 2001|
- Epstein-Barr virus
- Hodgkin's disease
- Latent membrane protein-1
- Wiskott-Aldrich syndrome