IgA deficiency is the most common human primary immune-deficiency. We evaluated the clinical and immunological characteristics of selective IgA deficiency in children in Israel. The study group included 63 children diagnosed with IgA deficiency from 1987 to 2005. Mean follow-up time per child was 10.6 years. Average age at diagnosis was 10.5 years. In one child, the IgA deficiency was transient. Infectious diseases, mainly recurrent pneumonia and ear infection, were common and occurred in 25 patients (39.7%). Allergic diseases were documented in 20 (31.7%) of our patients. Thirteen children (20.6%) had autoimmune diseases. Malignancies were diagnosed in three children (4.8%), an association that has not been reported in previous series. IgA deficiency appears to be a risk factor for infections, allergic diseases, autoimmune conditions, and malignancy.

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.