Hochmalignes Non-Hodgkin-Lymphom bei Hyper-IgE-Syndrom

Abstract

A largely symptom-free swelling of the right submandibular lymph nodes developed in a 23-year-old woman with hyper-IgE syndrome who had suffered from recurrent staphylococcal abscesses since childhood. Histological examination of the lymph nodes revealed highly malignant non-Hodgkin lymphoma of centroblastic type. Extensive staging tests did not reveal any further tumour manifestations. Because granulocyte chemotaxis is abnormal in hyper-IgE syndrome and the patient also had an infection of an old lung cyst, treatment was restricted to local radiotherapy of the cervical lymph nodes. Since 12 months there has been no recurrence. There is probably no connection between the hyper-IgE syndrome and the development of a malignant non-Hodgkin lymphoma, judging by the results of immunological and immunoelectrophoretic findings.

Hyperimmunoglobulin E syndrome: Two cases and a review of the literature

“…Multiple cases of lymphoma have been reported in HIES patients, including Hodgkin's, non-Hodgkin's, Burkitt's, and an unusual histiocytic variant. [45][46][47][48] The prevalence of autoimmune diseases is increased in failure of certain host defense mechanisms, most notably with a deficiency of complement or IgA, as is seen in HIES. 49 It has therefore been theorized that abnormal neutrophil chemotaxis places HIES patients at higher risk of not only infections, but also autoimmune diseases.…”