The RS score (Richter syndrome score), which is an estimate of the patients prognosis, is based on the patients performance status, LDH, platelet count, the size of the lymphoma tumors, and the number of prior therapies already received. Overall, the median survival is between five and eight months. Untreated, RS is invariably fatal. The Hodgkins lymphoma variant of Richters carries a better prognosis than the predominant diffuse large B-cell lymphoma type, but a worse prognosis than a de novo case of Hodgkins. Epidemiology
A SEER-based analysis estimated the risk of Richters transformation in Chronic Lymphocytic Leukemia (CLL) patients in the United States to be about 0.72%. The majority of transformations (85%) were Diffuse Large B-Cell Lymphoma (DLBCL), while transformation to Hodgkin occurred in 15% of cases. Most of those transformation events were nodal (74%). The most commonly involved extra-nodal sites were the GI tract (25%), skeletal system (19%), and the brain/CNS (12%). The median time from the diagnosis of CLL to the development of Richters transformation was about 4 years, with no significant difference in the time interval based on histology of the transformation event comparing DLBCL and Hodgkins. The median age at the time of Richters transformation was 66 years. References
Further reading
Tsimberidou AM, OBrien S, Khouri I, Giles FJ, Kantarjian HM, Champlin R, Wen S, Do KA, Smith SC, Lerner S, Freireich EJ, Keating MJ (May 20, 2006). "Clinical Outcomes and Prognostic Factors in Patients With Richters Syndrome Treated With Chemotherapy or Chemoimmunotherapy With or Without Stem-Cell Transplantation". Journal of Clinical Oncology. 24 (15): 2343–2351. doi:10.1200/JCO.2005.05.0187.
PMID 16710033. S2CID 25490586.