Richter transformation in 16 of 149 Chinese patients with chronic lymphocytic leukemia

Abstract

No confirmed risk factors are known to predict Richter syndrome (RS), and the value of clinical prognosticators conventionally applied to chronic lymphocytic leukemia (CLL) is not firmly established in this setting. The objective of this study was to present evidence for RS in Chinese patients with CLL and risk factors for CLL transformation to Richter syndrome. With a median follow-up of 43 months from CLL diagnosis in 149 Chinese patients, 16 (10.7%) patients progressed to diffuse large B-cell lymphoma (DLBCL). According to correlation analysis, a high level of lactate dehydrogenase (LDH) and CD38 positivity were found to be independent predictors of transformation to RS. Survival analysis showed that presence of RS, advanced Binet stage, high level of LDH, high level of β₂-microglobulin, high concentration of thymidine kinase (TK), ZAP-70 and CD38 expression, unmutated immunoglobulin heavy chain variable (IGHV) gene status and del(17p13) were adverse factors in determining overall survival (OS). Only del(17p13) was strongly associated with survival by multivariate Cox regression analysis. Median OS after transformation was 16 months (95% confidence interval, 5.6–26.4 months). The results support that RS is associated with a poor outcome, and a policy of close monitoring and careful biopsy is needed in patients carrying transformation risk factors.

Keywords::

• Richter syndrome
• chronic lymphocytic leukemia
• risk factors

Acknowledgements

This study was supported by the National Natural Science Foundation of China (30871104, 30971296, 81170485, 81170488), Natural Science Foundation of Jiangsu Province (BK2010584), Key Projects of the Health Department of Jiangsu Province (K201108), Jiangsu Province's Medical Elite Program (RC2011169), University Doctoral Foundation of the Ministry of Education of China (20093234110010), the Program for Development of the Innovative Research Team in the First Affiliated Hospital of NJMU, a Project Funded by the Priority Academic Program Development of Jiangsu Higher Education Institutions, and a Project of National Key Clinical Speciality.

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