Richter transformation of chronic lymphocytic leukaemia in the era of treatment with B-cell signalling pathway inhibitors
Abstract
Richter transformation (RT) is a severe complication of chronic lymphocytic leukaemia (CLL) affecting up to approximately 10% of patients with CLL. Most patients develop transformation to diffuse large B-cell lymphoma (DLBCL), which carries a dismal prognosis with a median survival of less than one year.
Clinical and laboratory features suggestive of RT include elevated lactate dehydrogenase, bulky or rapidly enlarging lymph nodes and B-symptoms. PET/CT scan can identify optimal site for excisional biopsy, which is a prerequisite for final RT diagnosis.
Chemoimmunotherapy remains the standard-of-care for RT, followed by stem cell transplantation in eligible patients. However, patients with RT are frequently elderly with comorbidities and poor performance status limiting intense chemotherapy or transplantation.
This review focuses on diagnosis, clinicopathological features, development and treatment of RT in the era of novel targeted agents.