Diffuse large B-cell lymphoma in a patient with hyper-IgE syndrome: successful treatment with risk-adapted rituximab-based immunochemotherapy

Abstract

Hyper IgE syndrome (HIES) is a rare primary immunodeficiency characterized by eczema, recurring skin and lung infections, elevated serum IgE and skeletal/connective tissue abnormalities. Here we report a case of a 44-year-old patient with diffuse large B-cell lymphoma successfully treated with risk-adapted rituximab-based immunochemotherapy.

Despite dose reductions and schedule adaptations required by severe pneumonia with sepsis occurring during the first cycle of R-CHOP immunochemotherapy, the patient achieved a long-lasting complete remission. 10.1016/j.leukres.2010.01.024

Keywords

• diffuse large B-cell lymphoma
• hyper-IgE syndrome
• successful treatment
• rituximab-based immunochemotherapy