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

Moradi, Soheila; Chavoshzadeh, Zahra; Izadyar, Mina; Mahjoub, Fatemeh; Rezaei, Nima
December 2009
Iranian Journal of Allergy, Asthma & Immunology;2009, Vol. 8 Issue 4, p215
Academic Journal
Idiopathic CD4+ Lymphocytopenia is a rare combined immunodeficiency disease, characterized by low CD4+ T-cell count and increased susceptibility to opportunistic infections, autoimmunity and malignancies after exclusion of secondary forms of CD4 lymphocytopenia. Here we present a 13-year old boy who was referred to our center because of destructive ulceration of soft and hard palates with extension to nose and maxillary sinus starting 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 studies revealed very low number of CD4+ T-cells (<100 cells/mm3). Secondary causes of CD4 lymphocytopenia, including HIV infection, were ruled out. Immunohistological studies of destructive lesions in oral and nasal cavity revealed angiocentric T-cell lymphoma. Unfortunately, the patient died in spite of treatment with a combination of irradiation and chemotherapy. This patient is the first reported case of lethal midline granuloma with origin T-cell lymphoma in idiopathic CD4+ lymphocytopenia.


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