Clinical presentation, outcome, and prognostic markers in patients with intravascular large B-cell lymphoma, a lymphoma study association (LYSA) retrospective study.
Cancer Med
; 11(19): 3602-3611, 2022 10.
Article
em En
| MEDLINE
| ID: mdl-35538643
ABSTRACT
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.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Linfoma Difuso de Grandes Células B
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Síndrome de Ativação Macrofágica
Tipo de estudo:
Diagnostic_studies
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Etiology_studies
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Observational_studies
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Prognostic_studies
Limite:
Adult
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Aged
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Aged80
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Humans
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Middle aged
Idioma:
En
Revista:
Cancer Med
Ano de publicação:
2022
Tipo de documento:
Article