PMID- 34151703 OWN - NLM STAT- MEDLINE DCOM- 20220414 LR - 20220414 IS - 1029-2403 (Electronic) IS - 1026-8022 (Linking) VI - 62 IP - 12 DP - 2021 Dec TI - Anaplastic lymphoma kinase-positive large B-cell lymphoma (ALK + LBCL): a systematic review of clinicopathological features and management. PG - 2845-2853 LID - 10.1080/10428194.2021.1941929 [doi] 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. FAU - Castillo, Jorge J AU - Castillo JJ AUID- ORCID: 0000-0001-9490-7532 AD - Division of Hematologic Malignancies, Dana-Farber Cancer Institute Harvard Medical School, Boston, MA, USA. FAU - Beltran, Brady E AU - Beltran BE AUID- ORCID: 0000-0003-4469-3817 AD - Department of Oncology and Radiotherapy, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru. FAU - Malpica, Luis AU - Malpica L AUID- ORCID: 0000-0002-7082-1846 AD - Division of Hematology and Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Marques-Piubelli, Mario L AU - Marques-Piubelli ML AUID- ORCID: 0000-0002-6324-2096 AD - Department of Translational Molecular Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Miranda, Roberto N AU - Miranda RN AUID- ORCID: 0000-0002-8467-5464 AD - Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. LA - eng PT - Journal Article PT - Systematic Review DEP - 20210621 PL - United States TA - Leuk Lymphoma JT - Leukemia & lymphoma JID - 9007422 RN - EC 2.7.10.1 (Anaplastic Lymphoma Kinase) RN - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases) SB - IM MH - Anaplastic Lymphoma Kinase MH - Humans MH - *Lymphoma, Large B-Cell, Diffuse/diagnosis/metabolism/therapy MH - *Lymphoma, Large-Cell, Anaplastic MH - Male MH - Phosphatidylinositol 3-Kinases MH - Prognosis MH - Receptor Protein-Tyrosine Kinases/genetics/metabolism OTO - NOTNLM OT - ALK OT - B-cell lymphoma OT - CD20-negative EDAT- 2021/06/22 06:00 MHDA- 2022/04/15 06:00 CRDT- 2021/06/21 08:47 PHST- 2021/06/22 06:00 [pubmed] PHST- 2022/04/15 06:00 [medline] PHST- 2021/06/21 08:47 [entrez] AID - 10.1080/10428194.2021.1941929 [doi] PST - ppublish SO - Leuk Lymphoma. 2021 Dec;62(12):2845-2853. doi: 10.1080/10428194.2021.1941929. Epub 2021 Jun 21.