Non-Hodgkin Lymphoma in Children and Adolescents: Progress Through Effective Collaboration, Current Knowledge, and Challenges Ahead

Abstract

Non-Hodgkin lymphoma is the fourth most common malignancy in children, has an even higher incidence in adolescents, and is primarily represented by only a few histologic subtypes. Dramatic progress has been achieved, with survival rates exceeding 80%, in large part because of a better understanding of the biology of the different subtypes and national and international collaborations. Most patients with Burkitt lymphoma and diffuse large B-cell lymphoma are cured with short intensive pulse chemotherapy containing cyclophosphamide, cytarabine, and high-dose methotrexate. The benefit of the addition of rituximab has not been established except in the case of primary mediastinal B-cell lymphoma. Lymphoblastic lymphoma is treated with intensive, semi-continuous, longer leukemia-derived protocols. Relapses in B-cell and lymphoblastic lymphomas are rare and infrequently curable, even with intensive approaches. Event-free survival rates of approximately 75% have been achieved in anaplastic large-cell lymphomas with various regimens that generally include a short intensive B-like regimen. Immunity seems to play an important role in prognosis and needs further exploration to determine its therapeutic application. ALK inhibitor therapeutic approaches are currently under investigation. For all pediatric lymphomas, the intensity of induction/consolidation therapy correlates with acute toxicities, but because of low cumulative doses of anthracyclines and alkylating agents, minimal or no long-term toxicity is expected. Challenges that remain include defining the value of prognostic factors, such as early response on positron emission tomography/computed tomography and minimal disseminated and residual disease, using new biologic technologies to improve risk stratification, and developing innovative therapies, both in the first-line setting and for relapse

Haematological cancers in African children: progress and challenges

“…The importance of intrathecal methotrexate and intrathecal cytarabine for central nervous system (CNS) prevention and treatment also became evident (Magrath, 2012b). In Europe and America, more intensive chemotherapy achieves 90% long-term survival (Minard-Colin et al, 2015). Such regimens may result in severe toxicities and treatment-related deaths due to lack of adequate supportive care in resource-constrained settings.…”

Lymphomas in childhood

“…NHL çocukluk çağı lenfomalarının %60'ını oluştu-rur ve en sık dördüncü çocukluk çağı malignensisidir (1,4,19) . Çoğunlukla hızlı büyüyen yüksek evre tümör-lerdir.…”

“…Bunlar içinde Burkitt lenfoma, NHL'ların %40'ını oluşturmaktadır. Burkitt lenfoma çoğunlukla abdominal kaynaklıdır ve hücre sayısı ikiye katlanma hızı en yüksek lenfomadır (2,19) . Çok hızlı büyüyen bu tümörde apoptozis de hızlıdır.…”

Anaplastic large cell lymphoma: pathology, genetics, and clinical aspects

“…The Children's Oncology Group is conducting a randomized phase II study to evaluate the feasibility and efficacy of combining BV or crizotinib with multi-agent chemotherapy (ALCL99 regimen) in pediatric newly diagnosed stage II to IV ALK-positive ALCL (NCT01979536). 213 Another randomized trial with risk stratification by minimal disseminated disease and anti-ALK antibody titers 158 is ongoing. The ALCL99 regimen with crizotinib or vinblastine will be evaluated in combination.…”

“…The ALCL99 regimen with crizotinib or vinblastine will be evaluated in combination. 213 In addition to crizotinib and ceritinib, alectinib is also in clinical evaluation for ALKpositive ALCL in Japan. Alectinib is a potent second-generation ALK inhibitor that is effective even in NSCLC harboring crizotinib-resistance mutations 214 and has demonstrated promising results in clinical settings.…”