Clinical presentation, outcome, and prognostic markers in patients with intravascular large B-cell lymphoma, a lymphoma study association (LYSA) retrospective study.
Adult
Aged
Aged, 80 and over
Antineoplastic Combined Chemotherapy Protocols
/ adverse effects
Cyclophosphamide
/ therapeutic use
Doxorubicin
/ therapeutic use
Humans
Lymphoma, Large B-Cell, Diffuse
/ diagnosis
Macrophage Activation Syndrome
/ drug therapy
Methotrexate
/ therapeutic use
Middle Aged
Prednisone
/ therapeutic use
Prognosis
Proto-Oncogene Proteins c-bcl-2
Retrospective Studies
Rituximab
/ therapeutic use
Vincristine
/ therapeutic use
Young Adult
BCL2 expression
autoimmune disorders
intravascular lymphoma
nodal involvement
Journal
Cancer medicine
ISSN: 2045-7634
Titre abrégé: Cancer Med
Pays: United States
ID NLM: 101595310
Informations de publication
Date de publication:
10 2022
10 2022
Historique:
revised:
03
12
2021
received:
06
09
2021
accepted:
23
12
2021
pubmed:
12
5
2022
medline:
14
10
2022
entrez:
11
5
2022
Statut:
ppublish
Résumé
Intravascular large B-cell lymphoma (lVLBCL) is a very rare type of large B-cell lymphoma. We conducted a retrospective study on IVLBCL patients treated from 2000 to 2016 in LYSA cooperative group centers. Sixty-five patients were identified in 23 centers. Median age at diagnosis was 69 years (range 23-92). Thirty-four patients (64%) had an IPI score >3 and 40 patients (67%) had a performance status ≥2. The most frequent extra-nodal locations were bone marrow (n = 34; 52%), central nervous system (n = 25; 39%), and skin (n = 21; 33%). Nodal involvement and endocrine system were observed in 34% (n = 22) and 18% (n = 12) of all cases, respectively. Twenty-six patients (41%) had macrophage activation syndrome. Tumor cells were frequently CD5 positive (52%) with a non-germinal center origin (86%). BCL2 was expressed in 87% of all samples analyzed (n = 20) and 43% of patients had a MYC/BCL2 double expression. Fifty-six patients were treated with a regimen of chemotherapy containing rituximab, among whom 73% reached complete remission. The median progression-free survival (PFS) and median overall survival (OS) were 29.4 months and 63.8 months, respectively. History of autoimmune disorder (Hazard ratio [HR] 3.3 [1.4-7.8]; p < 0.01), nodal involvement (HR 2.6 [1.4-5.1]; p < 0.01), lack of anthracycline (HR 0.1 [0-0.4] for use; p < 0.001), or no intensification at first-line regimen (p = 0.02) were associated with worse PFS. High-dose methotrexate use was not associated with better PFS or OS. Our study highlights the aggressive clinical picture of IVLBCL, in particular the frequency of macrophage activation syndrome, and the need for new therapies despite a response to R-CHOP-like regimen similar to non-intravascular diffuse large B-cell lymphomas.
Sections du résumé
BACKGROUND
Intravascular large B-cell lymphoma (lVLBCL) is a very rare type of large B-cell lymphoma.
METHODS
We conducted a retrospective study on IVLBCL patients treated from 2000 to 2016 in LYSA cooperative group centers.
RESULTS
Sixty-five patients were identified in 23 centers. Median age at diagnosis was 69 years (range 23-92). Thirty-four patients (64%) had an IPI score >3 and 40 patients (67%) had a performance status ≥2. The most frequent extra-nodal locations were bone marrow (n = 34; 52%), central nervous system (n = 25; 39%), and skin (n = 21; 33%). Nodal involvement and endocrine system were observed in 34% (n = 22) and 18% (n = 12) of all cases, respectively. Twenty-six patients (41%) had macrophage activation syndrome. Tumor cells were frequently CD5 positive (52%) with a non-germinal center origin (86%). BCL2 was expressed in 87% of all samples analyzed (n = 20) and 43% of patients had a MYC/BCL2 double expression. Fifty-six patients were treated with a regimen of chemotherapy containing rituximab, among whom 73% reached complete remission. The median progression-free survival (PFS) and median overall survival (OS) were 29.4 months and 63.8 months, respectively. History of autoimmune disorder (Hazard ratio [HR] 3.3 [1.4-7.8]; p < 0.01), nodal involvement (HR 2.6 [1.4-5.1]; p < 0.01), lack of anthracycline (HR 0.1 [0-0.4] for use; p < 0.001), or no intensification at first-line regimen (p = 0.02) were associated with worse PFS. High-dose methotrexate use was not associated with better PFS or OS.
CONCLUSIONS
Our study highlights the aggressive clinical picture of IVLBCL, in particular the frequency of macrophage activation syndrome, and the need for new therapies despite a response to R-CHOP-like regimen similar to non-intravascular diffuse large B-cell lymphomas.
Identifiants
pubmed: 35538643
doi: 10.1002/cam4.4742
pmc: PMC9554445
doi:
Substances chimiques
Proto-Oncogene Proteins c-bcl-2
0
Rituximab
4F4X42SYQ6
Vincristine
5J49Q6B70F
Doxorubicin
80168379AG
Cyclophosphamide
8N3DW7272P
Prednisone
VB0R961HZT
Methotrexate
YL5FZ2Y5U1
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
3602-3611Informations de copyright
© 2022 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.
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