Younger patients with Waldenström Macroglobulinemia exhibit low risk profile and excellent outcomes in the era of immunotherapy and targeted therapies.
Adenine
/ analogs & derivatives
Adult
Age Factors
Autografts
Disease-Free Survival
Female
Humans
Immunotherapy
Male
Middle Aged
Piperidines
Pyrazoles
/ administration & dosage
Pyrimidines
/ administration & dosage
Risk Factors
Stem Cell Transplantation
Survival Rate
Waldenstrom Macroglobulinemia
/ mortality
Journal
American journal of hematology
ISSN: 1096-8652
Titre abrégé: Am J Hematol
Pays: United States
ID NLM: 7610369
Informations de publication
Date de publication:
12 2020
12 2020
Historique:
received:
04
08
2020
accepted:
06
08
2020
pubmed:
12
8
2020
medline:
1
12
2020
entrez:
12
8
2020
Statut:
ppublish
Résumé
We analyzed 160 young Waldenström Macroglobulinemia (WM) patients with a median age of 49 years (range 23-55 years), diagnosed between January 2000 and January 2019 in 14 Italian centers. At diagnosis, 70% of patients were asymptomatic. With a median follow-up of 5.6 years, 57% have been treated. As initial therapy 79% of patients received chemo-immunotherapy, 13% a chemo-free induction and 8% chemotherapy only. At relapse or progression, 6% underwent an autologous stem cell transplantation. Overall, 19% of patients received ibrutinib during the course of the disease. According to IPSSWM, 63% were classified as low risk, 27% as intermediate risk and 10% as high risk. Five-year OS was shorter in high-risk as compared with low or intermediate risk patients (92.9% vs 100% P = .002). According to revised IPSSWM, 92% were classified as very low or low risk and 8% as intermediate risk, with a shorter 5-year OS in the latter group (87.5% vs 100%, P = .028). The OS of young WM patients was not significantly reduced as compared with age-matched, sex-matched and calendar year-matched general population. Early diagnosis, absence of high-risk features in symptomatic patients and high efficacy of modern treatments are the main determinants of the excellent outcome of young WM patients.
Substances chimiques
Piperidines
0
Pyrazoles
0
Pyrimidines
0
ibrutinib
1X70OSD4VX
Adenine
JAC85A2161
Types de publication
Clinical Trial
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1473-1478Informations de copyright
© 2020 Wiley Periodicals LLC.
Références
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