Angiocentric Nasal T-Cell Lymphoma in a Patient with Idiopathic CD4+ Lymphocytopenia

Idiopathic cd4+ lymphocytopenia is a rare combined immunodeficiency disease,characterized by low cd4+ t-cell count and increased susceptibility to opportunisticinfections, autoimmunity and malignancies after exclusion of secondary forms of cd4lymphocytopenia. here we present a 13-year old boy who was referred to our center becauseof destructive ulceration of soft and hard palates with extension to nose and maxillary sinusstarting at 6 months of age. he had a history of recurrent otitis media, chronic diarrhea,arthritis and herpetic lesions of eyes and mouth since the age of 5 years. laboratory studiesrevealed very low number of cd4+ t-cells (<100 cells/mm3). secondary causes of cd4lymphocytopenia, including hiv infection, were ruled out. immunohistological studies ofdestructive lesions in oral and nasal cavity revealed angiocentric t-cell lymphoma.unfortunately, the patient died in spite of treatment with a combination of irradiation andchemotherapy. this patient is the first reported case of lethal midline granuloma with origint-cell lymphoma in idiopathic cd4+ lymphocytopenia.