Richter's syndrome

Last reviewed 01/2018

Richter's syndrome is an aggressive large cell lymphoma which results from the transformation of an isolated lymph node in chronic lymphocytic leukaemia. The remaining lymph nodes are unaffected.

• around 10% of patients with CLL develop Richter's syndrome with transformation into an aggressive lymphoma, Hodgkin's lymphoma or prolymphocytic leukaemia (PLL)
• polychemotherapies with antibodies are a treatment option (but without long-lasting remission)
• allogeneic stem cell transplantation can be used as an experimental option in physically fit patients  (1)

Reference:

• (1) Eichhorst B et al. Chronic lymphocytic leukaemia: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2010;21 Suppl 5:v162-4