Diagnosis and management of lymphoblastic lymphoma in children, adolescents and young adults

William C Temple; Stephanie Mueller; Michelle L Hermiston; Birgit Burkhardt

doi:10.1016/j.beha.2023.101449

Diagnosis and management of lymphoblastic lymphoma in children, adolescents and young adults

Best Pract Res Clin Haematol. 2023 Mar;36(1):101449. doi: 10.1016/j.beha.2023.101449. Epub 2023 Feb 16.

Authors

William C Temple¹, Stephanie Mueller², Michelle L Hermiston³, Birgit Burkhardt²

Affiliations

¹ Paediatric Haematology and Oncology, University of California, San Francisco, USA; Paediatric Allergy, Immunology, and Bone Marrow Transplantation, University of California, San Francisco, USA.
² Paediatric Haematology and Oncology, University Hospital Muenster, Germany; NHL-BFM Study Center, University Hospital Muenster, Germany.
³ Paediatric Haematology and Oncology, University of California, San Francisco, USA; Paediatric Allergy, Immunology, and Bone Marrow Transplantation, University of California, San Francisco, USA. Electronic address: Michelle.hermiston@ucsf.edu.

PMID: 36907639
DOI: 10.1016/j.beha.2023.101449

Abstract

Lymphoblastic lymphoma (LBL) is the second most common type of non-Hodgkin Lymphoma (NHL) in children, adolescents, and young adults (CAYA), accounting for 25-35% of all cases. T-lymphoblastic lymphoma (T-LBL) comprises 70-80% of cases, while precursor B-lymphoblastic lymphoma (pB-LBL) makes up the remaining 20-25% of cases. Event-free and overall survival (EFS and OS) for paediatric LBL patients both exceed 80% with current therapies. Treatment regimens, especially in T-LBL with large mediastinal tumours, are complex with significant toxicity and long-term complications. Though prognosis overall is good for T-LBL and pB-LBL with upfront therapy, outcomes for patients with relapsed or refractory (r/r) disease remain dismal. Here, we review new understanding about the pathogenesis and biology of LBL, recent clinical results and future directions for therapy, and remaining obstacles to improve outcomes while reducing toxicity.

Keywords: Adolescents; CAR-T cells; Children; Immunotherapy; Lymphoblastic lymphoma; Novel agents; Precursor B cell; Precursor T cell.

Publication types

Review
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

MeSH terms

Adolescent
Child
Humans
Lymphoma, B-Cell*
Lymphoma, Non-Hodgkin*
Precursor Cell Lymphoblastic Leukemia-Lymphoma* / therapy
Precursor T-Cell Lymphoblastic Leukemia-Lymphoma* / therapy
Prognosis
Young Adult