Prognostic factors and treatment of pediatric acute lymphoblastic leukemia

Abstract

The event-free survival (EFS) for pediatric acute lymphoblastic leukemia (ALL) has shown remarkable improvement in the past several decades. In Korea also, a recent study showed 10-year EFS of 78.5%. Much of the improved outcome for pediatric ALL stems from the accurate identification of prognostic factors, the designation of risk group based on these factors, and treatment of appropriate duration and intensity according to risk group, done within the setting of cooperative clinical trials. The schema of first-line therapy for ALL remains mostly unchanged, although many groups have now reported on the elimination of cranial irradiation in all patients with low rates of central nervous system relapse. Specific high risk subgroups, such as Philadelphia chromosome-positive (Ph+) ALL and infant ALL continue to have significantly lower survival than other ALL patients. The introduction of tyrosine kinase inhibitors into therapy has led to enhanced outcome for Ph+ ALL patients. Infant ALL patients, particularly those with MLL rearrangements, continue to have poor outcome, despite treatment intensification including allogeneic hematopoietic cell transplantation. Relapsed ALL is a leading cause of mortality in pediatric cancer. Recent advances in immunotherapy targeting the CD19 of the ALL blast have shown remarkable efficacy in some of these relapsed and refractory patients. With improved survival, much of the current focus is on decreasing the long-term toxicities of treatment.

Clinical characteristics and analysis of treatment result in children with Ph‐positive acute lymphoblastic leukaemia in Poland between 2005 and 2017

“…Specific high‐risk subgroups, such as the molecular genetic abnormality, that is Philadelphia chromosome‐positive acute lymphoblastic leukaemia, continue to have significantly lower survival rates than other ALL children. Ph+ ALL was associated with a high relapse rate when treated with chemotherapy alone . Hematopoietic stem cell transplant (HSCT) was considered the therapy of choice, notably when a matched sibling donor (MSD) was available .…”

“…Ph+ ALL was associated with a high relapse rate when treated with chemotherapy alone. 12,13 Hematopoietic stem cell transplant (HSCT) was considered the therapy of choice, notably when a matched sibling donor (MSD) was available. 14 However, recent data have indicated that chemotherapy used together with tyrosine kinase inhibitor (TKI) may be an alternative effective therapy.…”

Bone morbidity in pediatric acute lymphoblastic leukemia

“…Acute lymphoblastic leukemia (ALL) is the most common cancer among pediatric and adolescent patients, and it accounts for major cancer-related deaths in childhood. 1) Various factors, including exogenous (e.g., infection) or endogenous exposures (e.g., inflammation, oxidative stress) and genetic background, contribute to disease onset; ALL occurs in roughly 1:2,000 children aged <15 years. 2) After successful evolution of chemotherapy and clinical trials spanning 5 decades, the current curability rate of ALL is approximately 90% in resource-rich countries.…”

Prevalence and Clinical Outcome of Philadelphia-Like Acute Lymphoblastic Leukemia: Systematic Review and Meta-analysis

“…ALL can also occur in adults, although less frequently, and the prognosis is far less favorable, with frequent relapse. 1 There are several factors responsible for the poor outcome of ALL in adults, including comorbidities, poor performance status, poor compliance, and higher frequency of high-risk genomic subgroups. 1 Philadelphia (Ph)-positive chromosome is a genetic translocation between chromosomes 9 and 22 that causes the production of a BCR-ABL1 fusion gene.…”