TY - JOUR
T1 - Richter's transformation in patients with chronic lymphocytic leukaemia
T2 - a Nationwide Epidemiological Study
AU - Ben-Dali, Yasmin
AU - Hleuhel, Mariam H
AU - da Cunha-Bang, Caspar
AU - Brieghel, Christian
AU - Poulsen, Christian B
AU - Clasen-Linde, Erik
AU - Bentzen, Hans H N
AU - Frederiksen, Henrik
AU - Christiansen, Ilse
AU - Nielsen, Linda H
AU - Enggaard, Lisbeth
AU - Helleberg, Marie
AU - Clausen, Michael
AU - Frederiksen, Mikael
AU - Pedersen, Robert S
AU - Niemann, Carsten U
AU - Andersen, Michael A
PY - 2020/6
Y1 - 2020/6
N2 - Richter's transformation (RT) refers to the development of an aggressive lymphoma in patients with chronic lymphocytic leukemia (CLL). Approximately, 2-10% of patients with CLL develop RT, most often as diffuse large B-cell lymphoma. To assess the incidence of RT, we examined risk factors for RT and death among patients with RT in a nationwide CLL cohort (from 2008 to 2016). Among 3772 patients, 113 had biopsy-proven RT. With a median follow-up of 4.3 years, the 5-year cumulative incidence of RT was 2.8%. Advanced Binet stage (B/C) (p<.001), unmutated IGHV (p<.001), and del(17p) (p<.001) were independently associated with risk of developing RT. Half of the patients with RT (49%) were treatment-naïve prior to transformation and demonstrated longer survival after RT compared to patients previously treated for CLL (6.1 vs. 2.8 years, p=.03). Whether this finding could be explained by a higher proportion of clonally unrelated RT among treatment-naïve patients, remain to be addressed.
AB - Richter's transformation (RT) refers to the development of an aggressive lymphoma in patients with chronic lymphocytic leukemia (CLL). Approximately, 2-10% of patients with CLL develop RT, most often as diffuse large B-cell lymphoma. To assess the incidence of RT, we examined risk factors for RT and death among patients with RT in a nationwide CLL cohort (from 2008 to 2016). Among 3772 patients, 113 had biopsy-proven RT. With a median follow-up of 4.3 years, the 5-year cumulative incidence of RT was 2.8%. Advanced Binet stage (B/C) (p<.001), unmutated IGHV (p<.001), and del(17p) (p<.001) were independently associated with risk of developing RT. Half of the patients with RT (49%) were treatment-naïve prior to transformation and demonstrated longer survival after RT compared to patients previously treated for CLL (6.1 vs. 2.8 years, p=.03). Whether this finding could be explained by a higher proportion of clonally unrelated RT among treatment-naïve patients, remain to be addressed.
KW - Chronic lymphocytic leukemia
KW - Hodgkin lymphoma
KW - Richter’s transformation
KW - diffuse large B-cell lymphoma
KW - prognostication
UR - http://www.scopus.com/inward/record.url?scp=85079449265&partnerID=8YFLogxK
U2 - 10.1080/10428194.2020.1719092
DO - 10.1080/10428194.2020.1719092
M3 - Journal article
C2 - 32031030
SN - 1042-8194
VL - 61
SP - 1435
EP - 1444
JO - Leukemia and Lymphoma
JF - Leukemia and Lymphoma
IS - 6
ER -