Author/Authors :
Moradi, Soheila Mofid Children Hospital - Shaheed Beheshti University, Tehran , Chavoshzadeh, Zahra Mofid Children Hospital - Shaheed Beheshti University, Tehran , Izadyar, Mina Department of Pediatrics - Pediatrics Center of Excellence - Children's Medical Center - Tehran University of Medical Sciences, Tehran , Mahjoub, Fatemeh Department of Pediatrics - Pediatrics Center of Excellence - Children's Medical Center - Tehran University of Medical Sciences, Tehran , Rezaei, Nima Department of Pediatrics - Pediatrics Center of Excellence - Children's Medical Center - Tehran University of Medical Sciences, Tehran
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/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.
