Hodgkin's disease in a child with hyperimmunoglobulin E syndrome

A 10-year-old boy with hyperimmunoglobulin E (HIE) syndrome was admitted to the hospital due to intermittent fever and a growing neck mass noted for 3 months. He had had chronic eczema and recurrent skin infections since infancy. At age 8, the diagnosis of HIE was established when a pneumatocele was found in the presence of extremely elevated serum IgE levels (7842 IU/mL). He also had defective T-lymphocyte function, manifested by cutaneus anergy, as well as abnormal proliferative response to mitogenic stimuli. Chemotactic function of neutrophils was normal. Pathological examination of the lymph node disclosed Hodgkin's disease (nodular sclerosis). A high index of suspicion for lymphoma should be given in patients with HIE syndrome who present with lymph node enlargement.