TY - JOUR
T1 - A population-based study of transformed marginal zone lymphoma
T2 - identifying outcome-related characteristics
AU - Bult, Johanna A. A.
AU - Huisman, Francien
AU - Zhong, Yujie
AU - Veltmaat, Nick
AU - Kluiver, Joost
AU - Tonino, Sanne H.
AU - Vermaat, Joost S. P.
AU - Chamuleau, Martine E. D.
AU - Diepstra, Arjan
AU - van den Berg, Anke
AU - Plattel, Wouter J.
AU - Brink, Mirian
AU - Nijland, Marcel
N1 - Funding Information: The authors would like to thank the registrars of the NCR for their dedicated data collection. The nationwide, population-based registry is maintained and hosted by the Netherlands Comprehensive Cancer Organization (IKNL). Publisher Copyright: © 2023, Springer Nature Limited.
PY - 2023/12/1
Y1 - 2023/12/1
N2 - Histological transformation of marginal zone lymphoma (tMZL) into diffuse large B-cell lymphoma is associated with poor outcomes. Clinical characteristics associated with transformation risk and outcome after transformation are largely unknown due to scarcity of data. In this population-based study, competing risk analyses were performed to elucidate clinical characteristics associated with developing transformation among 1793 MZL patients using the Netherlands Cancer Registry. Cox regression analyses were performed to elucidate clinical characteristics associated with risk of relapse and mortality after transformation. Transformation occurred in 75 (4%) out of 1793 MZL patients. Elevated LDH and nodal MZL subtype at MZL diagnosis were associated with an increased risk, and radiotherapy with a reduced risk of developing tMZL. Most tMZL patients received R-(mini)CHOP (n = 53, 71%). Age >60 years and (immuno)chemotherapy before transformation were associated with an increased risk of relapse and mortality after transformation. Two-year progression-free survival (PFS) and overall survival (OS) were 66% (95% CI 52–77%) and 75% (95% CI 62–85%) for R-(mini)CHOP-treated tMZL patients, as compared to a PFS and OS both of 41% (95% CI 19–63%) for patients treated otherwise. Our study offers comprehensive insights into characteristics associated with transformation and survival after transformation, thereby optimizing guidelines and patient counseling.
AB - Histological transformation of marginal zone lymphoma (tMZL) into diffuse large B-cell lymphoma is associated with poor outcomes. Clinical characteristics associated with transformation risk and outcome after transformation are largely unknown due to scarcity of data. In this population-based study, competing risk analyses were performed to elucidate clinical characteristics associated with developing transformation among 1793 MZL patients using the Netherlands Cancer Registry. Cox regression analyses were performed to elucidate clinical characteristics associated with risk of relapse and mortality after transformation. Transformation occurred in 75 (4%) out of 1793 MZL patients. Elevated LDH and nodal MZL subtype at MZL diagnosis were associated with an increased risk, and radiotherapy with a reduced risk of developing tMZL. Most tMZL patients received R-(mini)CHOP (n = 53, 71%). Age >60 years and (immuno)chemotherapy before transformation were associated with an increased risk of relapse and mortality after transformation. Two-year progression-free survival (PFS) and overall survival (OS) were 66% (95% CI 52–77%) and 75% (95% CI 62–85%) for R-(mini)CHOP-treated tMZL patients, as compared to a PFS and OS both of 41% (95% CI 19–63%) for patients treated otherwise. Our study offers comprehensive insights into characteristics associated with transformation and survival after transformation, thereby optimizing guidelines and patient counseling.
UR - http://www.scopus.com/inward/record.url?scp=85169518709&partnerID=8YFLogxK
U2 - https://doi.org/10.1038/s41408-023-00903-w
DO - https://doi.org/10.1038/s41408-023-00903-w
M3 - Article
C2 - 37658062
SN - 2044-5385
VL - 13
JO - Blood Cancer Journal
JF - Blood Cancer Journal
IS - 1
M1 - 130
ER -