Diffuse large B‐cell lymphoma (Richter syndrome) in patients with chronic lymphocytic leukaemia (CLL): a cohort study of newly diagnosed patients

• Published in: British journal of haematology 2013-09, Vol.162 (6), p.774-782
• Main Authors: Parikh, Sameer A., Rabe, Kari G., Call, Timothy G., Zent, Clive S., Habermann, Thomas M., Ding, Wei, Leis, Jose F., Schwager, Susan M., Hanson, Curtis A., Macon, William R., Kay, Neil E., Slager, Susan L., Shanafelt, Tait D.
• Format: Article
• Language: English
• Subjects: Adult; Aged; Aged, 80 and over; aggressive lymphoma; Biological and medical sciences; Cohort Studies; Disease Progression; Female; Hematologic and hematopoietic diseases; Humans; Leukemia, Lymphocytic, Chronic, B-Cell - complications; Leukemia, Lymphocytic, Chronic, B-Cell - drug therapy; Leukemia, Lymphocytic, Chronic, B-Cell - surgery; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma, Large B-Cell, Diffuse - complications; Lymphoma, Large B-Cell, Diffuse - drug therapy; Lymphoma, Large B-Cell, Diffuse - surgery; Male; Medical sciences; Middle Aged; Prognosis; purine analogues; Richter syndrome survival score; stem cell transplantation; Stem Cell Transplantation - methods; Survival Rate; transformation; Tumors; Young Adult
• ISSN: 0007-1048; 1365-2141
• DOI: 10.1111/bjh.12458

Summary Nearly all information about patients with chronic lymphocytic leukaemia (CLL) who develop diffuse large B‐cell lymphoma [Richter syndrome (RS)] is derived from retrospective case series or patients treated on clinical trials. We used the Mayo Clinic CLL Database to identify patients with newly diagnosed CLL between January 2000 and July 2011. Individuals who developed biopsy‐proven RS during follow‐up were identified. After a median follow‐up of 4 years, 37/1641 (2·3%) CLL patients developed RS. The rate of RS was approximately 0·5%/year. Risk of RS was associated with advanced Rai stage at diagnosis (P