TY - JOUR
T1 - Anaplastic lymphoma kinase-positive large B-cell lymphoma (ALK + LBCL)
T2 - a systematic review of clinicopathological features and management
AU - Castillo, Jorge J.
AU - Beltran, Brady E.
AU - Malpica, Luis
AU - Marques-Piubelli, Mario L.
AU - Miranda, Roberto N.
N1 - Publisher Copyright:
© 2021 Informa UK Limited, trading as Taylor & Francis Group.
PY - 2021
Y1 - 2021
N2 - 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.
AB - 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.
KW - ALK
KW - B-cell lymphoma
KW - CD20-negative
UR - http://www.scopus.com/inward/record.url?scp=85108295940&partnerID=8YFLogxK
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U2 - 10.1080/10428194.2021.1941929
DO - 10.1080/10428194.2021.1941929
M3 - Review article
C2 - 34151703
AN - SCOPUS:85108295940
SN - 1042-8194
VL - 62
SP - 2845
EP - 2853
JO - Leukemia and Lymphoma
JF - Leukemia and Lymphoma
IS - 12
ER -