Fatal histiocytic lymphoma of the brain associated with hyperimmunoglobulinemia-E and recurrent infections.
A 10-year-old girl with eczema, asthma, recurrent abscesses, middle ear infections and pneumonias developed a histiocytic lymphoma of the brain. Immunologic evaluation revealed hyperimmunoglobulinemia E, depressed neutrophil chemotaxis, absent delayed hypersensitivity skin test tests, low T-lymphocyte numbers and depressed in vitro lymphocyte responses. The occurrence in this patient of a histiocytic lymphoma, which is commonly associated with more classic immunodeficiency syndromes or immunosuppression for transplantation, suggests that individuals with hyperimmunoglobulinemia E and recurrent infections may also be at increased risk for developing unusual malignancies.
D001932:Brain Neoplasms / Q000150:complications*; D002648:Child; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications*; D007073:Immunoglobulin E; D007239:Infections / Q000150:complications*; D016403:Lymphoma, Large B-Cell, Diffuse / Q000150:complications*
D007073:Immunoglobulin E