Lethal midline granuloma

ABSTRACT

We report a case of lethal midline granuloma in a 12-year-old girl. She presented with epistaxis, nasal obstruction, and bilateral progressive proptosis. The respiratory tract and kidneys were clinically free, as revealed by chest X-ray films and abdominal sonography. Examination of the bone marrow showed hypercellularity, with increased plasma cells and marrow eosinophils. The blood picture was within normal limits. The diagnosis was confirmed by a CT scan of the skull. A biopsy from the nasopharynx revealed lymphocytes, reticulum cells, and atypical cells considered representative of polymorphic reticulosis. The chosen treatment in our case was a combination of radiotherapy and a course of chemotherapy consisting of endoxan, adriamycin, oncovine and prednisone. The short-term result of treatment was favorable