Anaplastic lymphoma kinase-positive large B-cell lymphoma (ALK + LBCL): a systematic review of clinicopathological features and management.
Leuk Lymphoma
; 62(12): 2845-2853, 2021 12.
Article
em En
| MEDLINE
| ID: mdl-34151703
ABSTRACT
Anaplastic lymphoma kinase-positive (ALK+) large B-cell lymphoma (LBCL) is a rare CD20-negative aggressive lymphoma. Given its rarity, data on ALK + LBCL are scarce and limited to case reports and small case series. Our systematic review included 184 unique cases published in the literature and shows that ALK + LBCL can affect individuals at any age, has a male predominance and is not associated with chronic viral infections. The malignant cells express ALK, VS38c, BLIMP-1, EMA, c-MYC, and BOB-1. The STAT3/STAT5, PI3K/AKT, PLCG2, and ERK pathways are important in the pathophysiology of ALK + LBCL. The prognosis of ALK + LBCL is poor with a 5-year survival rate of 28%. Early disease stage is associated with better outcomes. ALK inhibitors and other targeted agents could be of value in the treatment of ALK + LBCL. Additional research is needed to better understand, diagnose and treat ALK + LBCL.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Linfoma Difuso de Grandes Células B
/
Linfoma Anaplásico de Células Grandes
Tipo de estudo:
Diagnostic_studies
/
Prognostic_studies
/
Systematic_reviews
Limite:
Humans
/
Male
Idioma:
En
Revista:
Leuk Lymphoma
Assunto da revista:
HEMATOLOGIA
/
NEOPLASIAS
Ano de publicação:
2021
Tipo de documento:
Article
País de afiliação:
Estados Unidos