Large T-cell lymphoma in a 13-year-old girl with hyperimmunoglobulinemia E syndrome

Abstract

Large T-cell lymphoma was diagnosed in a 13-year-old girl with hyperimmunoglobulinemia E syndrome (HIES). Her past medical history included severe dermatitis, recurrent pneumonia, urinary tract infections, mucocutaneous herpetic infections, fungal skin infections, and staphylococcal sepsis. The diagnosis of HIES, based on the clinical features and a serum IgE level of >20000 IU/ml, was established when the girl was 6 years old. This is the eighth case of lymphoma in a patient with HIES reported in the English-language medical literature. HIES has not usually been considered a predisposing factor for malignancy, but in view of the rarity of HIES and the young age of the patients, this association seems to be more than coincidental. A link between lymphoma and Epstein-Barr virus (EBV) infection in patients with HIES has been proposed. Serological tests for EBV in our patient were positive 6 years prior to the development of the lymphoma; however, examination for EBV DNA in the lymph node biopsy failed to detect EBV in the tumor.

Pneumocystis jirovecii pneumonia in a baby with hyper-IgE syndrome

“…Fungal infections in HIES include mucocutaneous or disseminated candidiasis [10], histoplasmosis, and cryptococcosis. In addition, several malignancies, especially lymphomas, have been reported in HIES patients [8].…”

Occupational Exposure to High Molecular Weight Allergens and Lymphoma Risk Among Italian Adults

“…Epidemiologic evidence of an association between factors that compromise the immune system and lymphoma is robust (1-3); however, findings from research conducted to investigate the associations between allergic conditions and lymphoma have not provided clear evidence of an association, and no apparent mechanistic hypotheses have yet emerged. Case reports have described the occurrence of lymphomas among children and young adults with elevated immunoglobulin E (IgE) or hyper-IgE syndrome (4, 5), while findings from larger epidemiologic studies suggest that allergic disorders may be associated with a decreased risk for NHL (6-9), and in particular, that increasing IgE level may be associated with decreasing NHL risk (8-10). Overall, little is known about how IgE levels affect lymphoma risk and additional investigations are needed to further assess whether the risk may be associated with reactivity to one or more specific allergens.…”