Richter transformation in chronic lymphocytic leukemia (CLL)—a pooled analysis of German CLL Study Group (GCLLSG) front line treatment trials

• Published in: Leukemia Vol. 35; no. 1; pp. 169 - 176
• Main Authors: Al-Sawaf, O., Robrecht, S., Bahlo, J., Fink, A. M., Cramer, P., v Tresckow, J., Lange, E., Kiehl, M., Dreyling, M., Ritgen, M., Dürig, J., Tausch, E., Schneider, C., Stilgenbauer, S., Wendtner, C. M., Fischer, K., Goede, Hallek, M., Eichhorst, B.
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 01.01.2021; Nature Publishing Group
• Subjects: 692/699/1541/1990/283/1895; 692/699/67/1990/291/1621/1915; Adult; Aged; Aged, 80 and over; Autografts; B-cell lymphoma; Biopsy; Cancer; Cancer Research; Care and treatment; Cell Transformation, Neoplastic - genetics; Chronic lymphocytic leukemia; Clinical trials; Critical Care Medicine; Development and progression; Diagnosis; Disease Progression; Evaluation; Female; Genetic transformation; Genetic Variation; Germany; Hematology; Hodgkin's lymphoma; Humans; Intensive; Internal Medicine; Leukemia; Leukemia, Lymphocytic, Chronic, B-Cell - genetics; Leukemia, Lymphocytic, Chronic, B-Cell - pathology; Leukemia, Lymphocytic, Chronic, B-Cell - therapy; Lymphatic leukemia; Lymphocytes B; Lymphoma; Lymphoma - etiology; Lymphoma - mortality; Lymphoma - pathology; Lymphoma - therapy; Male; Medicine; Medicine & Public Health; Middle Aged; Neoplasm Grading; Neoplasm Staging; Oncology; Oncology, Experimental; Prognosis; Stem cell transplantation; Stem cells; Survival Analysis; Time Factors; Transplantation; Young Adult; Germany
• ISSN: 0887-6924; 1476-5551
• DOI: 10.1038/s41375-020-0797-x

Richter transformation (RT) is defined as development of aggressive lymphoma in patients (pts) with CLL. The incidence rates of RT among pts with CLL range from 2 to 10%. The aim of this analysis is to report the frequency, characteristics and outcomes of pts with RT enrolled in trials of the GCLLSG. A total of 2975 pts with advanced CLL were reviewed for incidence of RT. Clinical, laboratory, and genetic data were pooled. Time-to-event data, starting from time of CLL diagnosis, of first-line therapy or of RT diagnosis, were analyzed by Kaplan-Meier methodology. One hundred and three pts developed RT (3%): 95 pts diffuse large B-cell lymphoma (92%) and eight pts Hodgkin lymphoma (8%). Median observation time was 53 months (interquartile range 38.1–69.5). Median OS from initial CLL diagnosis for pts without RT was 167 months vs 71 months for pts with RT (HR 2.64, CI 2.09–3.33). Median OS after diagnosis of RT was 9 months. Forty-seven pts (46%) received CHOP-like regimens for RT treatment. Three pts subsequently underwent allogeneic and two pts autologous stem cell transplantation. Our findings show that within a large cohort of GCLLSG trial participants, 3% of the pts developed RT after receiving first-line chemo- or chemoimmunotherapy. This dataset confirms the ongoing poor prognosis and high mortality associated with RT.