TY - JOUR
T1 - Survival trends in young patients with Waldenström macroglobulinemia
T2 - Over five decades of experience
AU - Chohan, Karan L.
AU - Paludo, Jonas
AU - Vallumsetla, Nishanth
AU - Larson, Dirk
AU - King, Rebecca L.
AU - He, Rong
AU - Gonsalves, Wilson
AU - Inwards, David
AU - Witzig, Thomas E.
AU - Swaika, Abhisek
AU - Jain, Tania
AU - Leung, Nelson
AU - Ailawadhi, Sikander
AU - Reeder, Craig B.
AU - Lacy, Martha Q.
AU - Rajkumar, S. Vincent
AU - Kumar, Shaji
AU - Kyle, Robert A.
AU - Gertz, Morie A.
AU - Ansell, Stephen M.
AU - Kapoor, Prashant
N1 - Publisher Copyright:
© 2023 Wiley Periodicals LLC.
PY - 2023/3
Y1 - 2023/3
N2 - Waldenström macroglobulinemia (WM) is a rare, indolent lymphoma, that predominately affects the elderly. We report the outcomes of young WM patients, evaluated over five decades, compared to their older counterparts, matched for the time of diagnosis. Between January 1, 1960 and October 31, 2013, 140 (11.8%) WM patients were ≤50 years of age at diagnosis in our database, and their estimated 10-year overall survival (OS) was 74%, with death attributable to WM in a higher proportion of patients compared to their older (≥65 years) counterparts (91% vs. 58%, p =.0001). Young patients were grouped into three cohorts based on the timing of the initiation of therapy: Group 1 (1960–1977, n = 12), Group 2 (1978–1995, n = 48), and Group 3 (1996–2013, n = 74). Among young patients, there was no disease-specific survival (DSS) difference across the three periods, [median DSS at 13 years (95% CI 5–23), 16 years (95% CI 14–22), and 15 years (95% CI 10-NR; p =.41), respectively]. However, DSS for the older cohort incrementally improved (Group 1, median 5.2 years, Group 2: 9.6 years, Group 3: 12 years; p =.05) over these periods. The estimated average years-of-life lost for the young cohort was 11.2 years from diagnosis, based on the expected survival for a normal age- and sex-matched population. Despite a protracted disease course, nearly all young patients succumb to their disease. In contrast to the improved survival of the elderly patient population, the evolving treatment strategies in WM have not impacted the outcome of young patients; however, the impact of Bruton tyrosine kinase inhibitors on this unique patient population remains to be determined.
AB - Waldenström macroglobulinemia (WM) is a rare, indolent lymphoma, that predominately affects the elderly. We report the outcomes of young WM patients, evaluated over five decades, compared to their older counterparts, matched for the time of diagnosis. Between January 1, 1960 and October 31, 2013, 140 (11.8%) WM patients were ≤50 years of age at diagnosis in our database, and their estimated 10-year overall survival (OS) was 74%, with death attributable to WM in a higher proportion of patients compared to their older (≥65 years) counterparts (91% vs. 58%, p =.0001). Young patients were grouped into three cohorts based on the timing of the initiation of therapy: Group 1 (1960–1977, n = 12), Group 2 (1978–1995, n = 48), and Group 3 (1996–2013, n = 74). Among young patients, there was no disease-specific survival (DSS) difference across the three periods, [median DSS at 13 years (95% CI 5–23), 16 years (95% CI 14–22), and 15 years (95% CI 10-NR; p =.41), respectively]. However, DSS for the older cohort incrementally improved (Group 1, median 5.2 years, Group 2: 9.6 years, Group 3: 12 years; p =.05) over these periods. The estimated average years-of-life lost for the young cohort was 11.2 years from diagnosis, based on the expected survival for a normal age- and sex-matched population. Despite a protracted disease course, nearly all young patients succumb to their disease. In contrast to the improved survival of the elderly patient population, the evolving treatment strategies in WM have not impacted the outcome of young patients; however, the impact of Bruton tyrosine kinase inhibitors on this unique patient population remains to be determined.
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U2 - 10.1002/ajh.26807
DO - 10.1002/ajh.26807
M3 - Article
C2 - 36588384
AN - SCOPUS:85145414783
SN - 0361-8609
VL - 98
SP - 432
EP - 439
JO - American journal of hematology
JF - American journal of hematology
IS - 3
ER -