Transformation of chronic lymphocytic leukemia (CLL) to an aggressive lymphoma, so-called Richter syndrome, usually includes diffuse large B-cell lymphoma and classic Hodgkin lymphoma. The transformation can be clonally related to the underlying CLL and is often Epstein-Barr virus (EBV) associated. Here we report the case of an 86-year-old woman with a newly identified CLL-like monoclonal B-lymphocytosis who developed diffuse lymphadenopathy. Biopsy of the left axillary lymph node showed EBV-positive large B-cell lymphoma with morphologic and immunophenotypic features intermediate between diffuse large B-cell lymphoma and classic Hodgkin lymphoma, so-called gray zone lymphoma. Comprehensive immunophenotypic, cytogenetic, and molecular studies demonstrate a clonal relatedness that suggests a transformation from monoclonal B-lymphocytosis to EBV+ gray zone lymphoma.
From the Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY.
Reprints: Wenbin Xiao, MD, PhD, Department of Pathology, Hematopathology Service, Memorial Sloan Kettering Cancer Center, 1275 York Ave, New York, NY 10065. E-mail: firstname.lastname@example.org.
Contributions: W.X. and Y.L. conceived the study, collected and analyzed the data, and wrote the manuscript. C.H. and M.A. annotated the sequencing data. M.R. and A.D. interpreted data. J.B. coordinated the project. Y.Z. performed and reviewed cytogenetic studies. All the authors approved the final version of the manuscript.
This study was supported in part through the National Institutes of Health/National Cancer Institute Cancer Center support grant P30 CA008748. W.X. is supported by a startup fund from the Department of Pathology at Memorial Sloan Kettering Cancer Center.
The authors have no conflicts to declare.