8/25/2023 0 Comments Prognostic factor![]() ![]() In contrast, the rare patients with E2A-HLF (t(17 19)(q22 p13.3) rearrangement have extremely poor outcome 18). However, patients treated on intensive, contemporary therapy were found to have favorable outcome 17), and this genetic abnormality is no longer deemed a risk factor by several treatment groups. Patients with E2A-PBX1 (t(1 19)(q23 p13.3)) had previously been considered to have poor prognosis. Hence, testing for TP53 mutations in patients with low hypodiploidy may allow for genetic counseling for those with germline mutations. A recent study found that 91.2% patients with low hypodiploidy have TP53 mutations, many of whom also showed the mutations in nontumor cells, indicating that ALL patients with low hypodiploidy may have underlying Li-Fraumeni syndrome 16). In contrast, hypodiploid ALL patients with ≤44 chromosomes, including those with low hypodiploidy (32–39 chromosomes) and near haploidy (24–31 chromosomes), have significantly worse survival 15). Most patients with hypodiploid ALL have 45 chromosomes and their outcome has been reported to be similar to those with nonhypodiploid ALL 14). MLL rearrangements are found in about 75% of infant ALL patients and predict a dismal outcome for this group of patients. Recurrent genetic abnormalities associated with poor prognosis include BCR-ABL1 (t(9 22)(q34 q11.2)), that is Philadelphia chromosome-positive (Ph+) ALL, rearrangements of MLL (or KMT2A)(11q23), and hypodiploidy. In our institution, we found that ETV6-RUNX1(+) ALL patients who were MRD(+) at the end of remission induction had EFS of 33%, in contrast to the 91% EFS for those who were MRD(-) 13). However, even within this favorable outcome subgroup, patients with detectable minimal residual disease (MRD) at the end of remission induction may have significantly lower survival than those who are MRD(-) 12). Those that predict excellent prognosis include ETV6-RUNX1 (t(12 21)(p13 q22)) and high hyperdiploidy, that is a chromosome number ≥51 10, 11). Genetic abnormalities of the leukemic blast, including aneuploidy and recurrent translocations and deletions, are important factors in the determination of risk group and outcome in Pre-B ALL. ![]() Current focus is on further understanding the biology of the disease and developing novel therapeutics in order to salvage patients who relapse or remain refractory to first-line treatment, as well as on minimizing the long-term adverse effects of chemotherapy. Much of the improvement in survival stems from the classification of patients into risk groups based on prognostic factors, and the adjustment of treatment intensity according to risk group, within the setting of national and multinational clinical trials. In Korea also, we reported a 10-year event-free survival (EFS) of 78.5% and OS of 81.9% for a large number of patients treated on CMCP-ALLL2005 and -ALL2008 regimens 6). Whereas 50 years ago the survival for pediatric ALL was 10%–20% 2), currently long-term overall survival (OS) rates are 80%–90% 3, 4, 5). In Korea, the age-standardized incidence rate of ALL is approximately 28 patients per million in the 0–14 year old age group 1). With improved survival, much of the current focus is on decreasing the long-term toxicities of treatment.Īcute lymphoblastic leukemia (ALL) is the most common cancer in the pediatric age group and is responsible for the most cancer-related deaths in children and adolescents. Recent advances in immunotherapy targeting the CD19 of the ALL blast have shown remarkable efficacy in some of these relapsed and refractory patients. Relapsed ALL is a leading cause of mortality in pediatric cancer. Infant ALL patients, particularly those with MLL rearrangements, continue to have poor outcome, despite treatment intensification including allogeneic hematopoietic cell transplantation. The introduction of tyrosine kinase inhibitors into therapy has led to enhanced outcome for Ph+ ALL patients. 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 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. 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. In Korea also, a recent study showed 10-year EFS of 78.5%. The event-free survival (EFS) for pediatric acute lymphoblastic leukemia (ALL) has shown remarkable improvement in the past several decades.
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