title: Angiocentric nasal T-cell lymphoma in a patient withidiopathic CD4+ lymphocytopenia.
abstract: 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/mm(3)). 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.
authors.affiliation: Mofid Children Hospital, Shaheed Beheshti University, Tehran, Iran.
authors.forename: Soheila
authors.initials: S
authors.lastname: Moradi
authors.forename: Zahra
authors.initials: Z
authors.lastname: Chavoshzadeh
authors.forename: Mina
authors.initials: M
authors.lastname: Izadyar
authors.forename: Fatemeh
authors.initials: F
authors.lastname: Mahjoub
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
mesh_terms: D000293:Adolescent; D018791:CD4 Lymphocyte Count; D015496:CD4-Positive T-Lymphocytes / Q000473:pathology*; D006801:Humans; D016399:Lymphoma, T-Cell / Q000097:blood / Q000150:complications / Q000473:pathology; D008231:Lymphopenia / Q000097:blood / Q000150:complications / Q000473:pathology; D008297:Male; D009669:Nose Neoplasms / Q000097:blood / Q000150:complications / Q000473:pathology