Lymphoblastic lymphoma in children and adolescents: review of current challenges and future opportunities

Abstract

Lymphoblastic lymphoma (LBL) is the second most common type of Non-Hodgkin Lymphoma (NHL) in childhood and adolescence, accounting for 25-35% of all cases. The majority, 70-80%, is of T-lymphoblastic origin while 20-25% arise from B lymphoblasts. With current therapy, the event-free and overall survivals for paediatric LBL patients now exceeds 80%. Therapy, especially in T-LBL with large mediastinal tumours, is challenging, with both significant morbidity and late sequela. An additional challenge is the dismal prognosis of patients with refractory or relapsed disease. This review article will focus on the growing knowledge of the pathogenesis and biology of LBL, recent advances and challenges in the therapy of LBL, and ongoing and future efforts and opportunities in optimizing therapy and developing novel targeted treatment approaches.

Effect of SLCO1B1 Polymorphisms on High‐Dose Methotrexate Clearance in Children and Young Adults With Leukemia and Lymphoblastic Lymphoma

“…In our study, most patients (n = 96; 360 courses) received HD MTX at 5 g/m 2 , which is the dosing regimen utilized most frequently in contemporary COG protocols for the treatment of a large majority of pediatric oncology patients in the United States. We confirmed an association between the key SLCO1B1 variant 521T>C and reduced HD MTX clearance at a dose of 5 g/m 2 .…”

B‐cell lymphoblastic lymphoma with cutaneous involvement and a KMT2A gene rearrangement

“…Debate still exists about whether LBL and acute lymphoblastic leukemia (ALL) are distinct entities or the same disease at a molecular level with a different clinical presentation. 1 According to the WHO classification, 2 LBL is distinguished from ALL by a bone marrow blast count of less than 20% of nucleated cells. The majority of lymphoblastic lymphomas originate from the T-cell lineage and occur in children.…”

The effect of graft‐versus‐host disease on outcomes after allogeneic stem cell transplantation for refractory lymphoblastic lymphoma in children and young adults

“…2,3,15 Although recently developed agents, including kinase inhibitors and immune therapies (e.g., chimeric antigen receptor-T cells), are promising for B-cell non-Hodgkin lymphoma, there is no such treatment for T-LBL that accounts for approximately 80% of LBL. 16,17 A more efficient method of allogeneic SCT that can achieve a full graft-versus-lymphoma effect should be investigated in a carefully planned clinical study.…”