Richter's Syndrome manifested as diffuse large B-cell lymphoma of the mandible with lytic lesions and hypercalcemic crisis

Abstract

Richter's Syndrome (RS) is a transformation from chronic lymphocytic leukemia (CLL) into more aggressive lymphoma. RS occurs in about 5% of patients with CLL and its clinical outcome is poor. Extranodal involvements of RS may be present in up to 40% of patients and central nervous system manifestation was found to be the most common. Mandibular localization of RS has not been reported so far. There is no established standard treatment for RS. The improvement of survival in RS patients is achievable with intensive anti-lymphoma chemotherapy and subsequent allogeneic stem cell transplantation (alloHSCT) performed in complete remission. Herein we report a female with RS of the right mandible and hypercalcemia was its first clinical manifestation.