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INTRODUCTION

SUMMARY

Diffuse large B-cell lymphoma (DLBCL) is the most common lymphoma diagnosis globally. The term DLBCL encompasses several distinct subtypes of lymphoma that differ by histologic features, molecular drivers, epidemiologic risk factors, and at times, treatment selection. The common unifying feature is the presence of aggregates of large, malignant centroblast B lymphocytes. Although DLBCL occurs at all ages, the incidence increases with age and peaks in the seventh decade of life. The typical clinical presentation is with a rapidly enlarging lymph nodes, although extranodal involvement occurs frequently. Systemic symptoms occur in a substantial subset of patients and include fever, sweats, and weight loss. The goal of treatment is cure, which is achievable in approximately two-thirds of patients with combination chemoimmunotherapy. The mainstay of therapy is cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab (R-CHOP) given every 21 days for six cycles. Early-stage disease may also be managed with fewer cycles of chemoimmunotherapy with or without radiotherapy. In patients who relapse, salvage chemotherapy at standard doses is rarely curative. High-dose chemotherapy and autologous stem cell transplantation cure a significant minority of patients who have relapsed disease. Chimeric antigen receptor T cells targeting the cluster of differentiation-19 (CD19) antigen have efficacy as a third-line or subsequent therapy and are being evaluated as an alternative to autologous stem cell transplantation in first relapse in prospective clinical trials.

Acronyms and Abbreviations

ABC, activated B-cell–like; ACVBP, doxorubicin (Adriamycin), cyclophosphamide, vindesine, bleomycin, prednisone; BCL2, B-cell lymphoma 2 gene, BCL6, B-cell lymphoma 6 gene; BCR, B-cell receptor; BEAC, BCNU, etoposide, cytosine arabinoside, cyclophosphamide; BR, bendamustine and rituximab; CALGB, Cancer and Leukemia Group B; CAR-T cells, chimeric antigen receptor T cells; CHOP, cyclophosphamide, doxorubicin, vincristine, prednisone; CNS, central nervous system; COO, cell of origin; CR, complete remission; CT, computed tomography; CytaBOM, cytarabine, bleomycin, vincristine, methotrexate (with leucovorin rescue); DA-EPOCH-R, dose-adjusted EPOCH with rituximab; DEL, double expressor lymphoma; DFS, disease-free survival; DHAP, dexamethasone, high dose cytosine arabinoside, cisplatin; DHL, double hit lymphoma; DLBCL, diffuse large B-cell lymphoma; EBER-ISH, Epstein-Barr encoding region in-situ hybridization; EBV, Epstein-Barr virus; ECOG, Eastern Cooperative Oncology Group; EFS, event-free survival; EPOCH, etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin; ESHAP, etoposide, methylprednisolone, cytarabine, cisplatin; FDG, 18-fluorodeoxyglucose; FISH, fluorescent in situ hybridization; GCB, germinal center B-cell–like; GDP, gemcitabine, dexamethasone, cisplatin; GELA, Groupe d’Etude des Lymphomes de l’Adulte; GEP, gene expression profiling; GnRH, gonadotrophin releasing hormone; HAART, highly active anti-retroviral therapy; HBV, hepatitis B virus; HCV, hepatitis C virus; HGBL, high grade B-cell lymphoma; HHV8, human herpes virus 8; HIV, human immunodeficiency virus; ICE, ifosfamide, carboplatin, etoposide; IFRT, involved-field radiation therapy; IPI, international prognostic index; IRAK. IL-1 receptor associated kinase; IV, intravenously; IVLBCL, intravascular large B-cell lymphoma; LDH, lactate dehydrogenase; LYSA, Lymphoma Study Association; m-BACOD, moderate-dose methotrexate, bleomycin, doxorubicin, cyclophosphamide, vincristine, dexamethasone; MACOP-B, high-dose methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisone, bleomycin; MRI, magnetic resonance imaging; NCI, National Cancer Institute; NCTN, National Clinical Trials Network; NHL, non-Hodgkin lymphoma; NKκB, nuclear factor kappa B; NOS, not otherwise specified; ORR, overall response rate; OS, ...

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