Orbital Richter Syndrome

Abstract

We report two patients with previously diagnosed chronic lymphocytic leukemia who developed Richter syndrome in the orbit as the sole extranodal site. The medical history, clinical findings, orbital imaging and histopathological features of the patients were reviewed. Treatment protocols and the outcomes were also assessed. The first patient developed Richter syndrome at the age of 64 years, 3 years after the diagnosis of chronic lymphocytic leukemia. The tumor was located at the inferotemporal quadrant of the orbit. The second patient was 59 years old when Richter syndrome arose in the lacrimal gland, 4 years after the diagnosis of chronic lymphocytic leukemia. Incisional biopsy from the orbital tumors were performed. Histopathological findings included diffuse CD20, CD 23, CD5, bcl2, bcl6 positive lymphocytic infiltration. Both patients were treated with chemotherapy and rituximab. During 3 years of follow-up, there was no orbital or systemic recurrence of the disease. Richter syndrome may develop in the orbital soft tissue and the lacrimal gland, and the orbital disease appears to have a better prognosis compared to patients with systemic involvement.

• Chronic lymphocytic leukemia
• lacrimal gland
• orbit
• Richter syndrome
• rituximab