New developments in Richter syndrome

摘要

Riehter syndrome refers to the transformation of chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma to an aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL), a type of non-Hodgkin lymphoma. There are also rare cases in which a transformation to Hodgkin lymphoma has been described. Riehter syndrome occurs in approximately 5% of patients with CLL, and the average time from diagnosis of CLL to transformation is approximately 2 to 5 years. Originally described in 1928 by Dr Maurice Riehter, Riehter syndrome is typically characterized by worsening adenopathy, the development or worsening of B symptoms, and rising levels of lactate dehydrogenase (LDH). Prognosis is typically poor, with a median survival of only 8 to 12 months.