Richter transformation in Chronic Lymphocytic Leukemia

Chronic lymphocytic leukemia can evolve to an aggressive lymphoma-in most of the cases diffuse large B cells lymphoma, rarely Hodgkin lymphoma-and this complication is defined Richter syndrome (RS). Immunogenotypic features that characterize RS include unmutated IgHV status with high prevalence of IgHV4-39/D6-13/J5 sequence; deletion of chromosome 17p or 11q; activation of oncogenes as NOTCH1 and c-MYC; inactivation of onco-suppressors as TP53 and CDKN2A; high expression of CD38 in lymph-nodes. The prognosis of this condition is very poor: patients experience a rapid clinical deterioration with frequent therapeutic failure since the current options include suboptimal strategies as standard chemo-immunotherapy followed by hematopoietic stem cells transplantation or enrollment in clinical trials which investigate the efficacy of target drugs. Understanding the biology of such a heterogeneous condition is crucial to personalize the treatment and improve patient's survival.

Automated summary

Chronic lymphocytic leukemia can transform into an aggressive lymphoma called Richter syndrome. Immunogenotypic features of Richter syndrome include unmutated IgHV status, deletion of chromosome 17p or 11q, activation of oncogenes, and inactivation of onco-suppressors. The prognosis for patients with Richter syndrome is very poor, and current treatment options are limited. Understanding the biology of this condition is crucial for personalized treatment and improving survival.