Nasal T-cell lymphoma as a type of so-called "lethal midline granuloma"

Abstract

Six cases were described in which an initial clinical diagnosis of "rhinitis gangrenosa progressiva" or lethal midline granuloma was made. The histological examinations of their surgical and autopsy specimens proved that their nasologic diseases could all be identified as malignant lymphoma arising from the nasal cavity, showing the general histologic characteristics reported for T-cell lymphomata derived from peripheral T-cells. This histologic observation was then confirmed by immunofluorescence studies using various antisera directed toward either human T- or B-cell-surface antigens. These studies clearly demonstrated that their malignant cells bore human Ly-l-like antigen but lacked human TL-like and Ia-like antigens as well as surface-bound immunoglobulins, indicating their peripheral T-cell origin. These data may suggest that so-called "rhinitis gangrenosa progressiva" or lethal midline granuloma contains at least two distinct disease categories, one of which is Wegener's granulomatosis, and the other of which is nasal T-cell lymphoma as described herein.