UVEAL INVOLVEMENT IN SYSTEMIC ANGIOTROPIC LARGE-CELL LYMPHOMA - MICROSCOPIC AND IMMUNOHISTOCHEMICAL STUDIES

Background: Angiotropic large cell lymphoma is a rare, generally fatal disease characterized by multifocal proliferation of neoplastic mononuclear cells within the lumens of blood vessels. Methods: The authors report the clinical and immunohistochemical features of four patients with angiotropic large cell lymphoma. Results: All patients presented with central nervous system symptoms, and three of the four had fever of unknown origin and anemia. The diagnosis was established by postmortem examination of the eyes in four patients and additionally by autopsy in three of the four patients. Two of the three autopsied patients had rare foci of extravascular involvement. One patient had erythrophagocytosis. Immunohistochemical stains on paraffin-embedded sections confirmed the diagnosis of lymphoma in all four patients. Conclusion: The results of this study support the B-cell lymphocyte origin of angiotropic large cell lymphoma.