Purpose
Children with Down syndrome (DS) show a higher risk of acute leukemia than those without DS. In this study, we investigated the nationwide incidence of acute leukemia among children with DS and compared their epidemiologic characteristics with those of children with acute leukemia but without DS.
Materials and Methods
Using the National Health Insurance Service database, we selected patients with acute leukemia aged 0–19 years at diagnosis between 2007 and 2016.
Results
Among the 4,697 children with acute leukemia, 54 (1.1%) had DS. The median incidence rate of leukemia with DS by year was 1.3% (range, 0.2%–2.0%). Sixteen patients with acute lymphoblastic leukemia (ALL; 29.6%) and 36 with acute myeloid leukemia (AML; 66.7%) had DS. The DS group showed younger age at diagnosis than the non-DS group, and diagnosis of AML was more frequent in the DS group than in the non-DS group (3 years vs. 9 years, p<0.001; 66.7% vs. 32.4%, P<0.001, respectively). The 5-year overall survival was comparable between the DS and non-DS groups (88.0% vs. 81.9%, p=0.375). Among all the Koreans born between 2007 and 2008, the incidences of acute leukemia, ALL, and AML were 49.25, 20.75, and 163.38 times higher, respectively, in the DS group than in the non-DS group.
Conclusion
Our findings support the fact that the incidence of acute leukemia is higher among patients with DS than among those without DS in Korea. However, the DS and non-DS groups in this study had a comparable overall survival rate.
Citations
Citations to this article as recorded by
Does Down syndrome affect the prognosis of acute lymphoblastic leukemia? A systematic review and meta-analysis Francisco Cezar Aquino de Moraes, Artur de Oliveira Macena Lôbo, Vitor Kendi Tsuchiya Sano, Caroline R. M. Pereira, Lucyana Barbosa Cardoso Leão, Thiago Xavier Carneiro, Rommel Mario Rodríguez Burbano Expert Review of Hematology.2025; 18(11): 961. CrossRef
Prevalência de neoplasias malignas em pessoas com síndrome de Down: uma revisão de escopo Válery Muniz de Sousa, Mônica Vilela Heimer, Danilo Bastos Moreno, Sandra Conceição Maria Vieira Caderno Pedagógico.2025; 22(13): e21693. CrossRef
Cancer risks related to intellectual disabilities: A systematic review Amina Banda, Jenneken Naaldenberg, Aura Timen, Agnies van Eeghen, Geraline Leusink, Maarten Cuypers Cancer Medicine.2024;[Epub] CrossRef
Objectives Colony stimulating factors have been shown to accelerate recovery from severe neutropenia after intensive chemotherapy. To prove its clinical effectiveness, we conducted controlled study to administer G-CSF in acute leukemia after induction chemotherapy. Methods: Forty patients with newly diagnosed and relapsed or refractory acute leukemia after remission induction chemotherapy were randomly assigned to one of two groupa (20 G- CSF treated group, 20 control grouP). Treatment with G-CSF(200 ug/m(2)/d) was started 48 hours after the end of chemotherapy and continued until the neutrophil count rose above 1,500 /mm(3). Results: Treatment with G-CSF shortened neutropenic period after chemotherapy, i.e., median duration of neutrophil counts less than 1,000/mm(3) was 21 days in control group and 12 days in G-CSF treated group. The incidence of infection was 80%in control group, 70% in G-CSF treated group, but it did not show any statistically significant difference. Microbiologically documented infection was 40% in control group, 20% in G-CSF treated group. Febrile periods and duration of antibiotic administration were decreased to 7 days and 18 days respectively. In G- CSF treated group, two Patients reported mild bone pain. There was no evidence that G-CSF could increase remission duratian and survival. Conclusion: It appears that recombinant G-CSF is safe and useful in neutropenic patients after intensive chemotherapy, accelerating neutrophil recovery and thereby reducing the incidence of documented infection and duration of antibiotic ad#ministration.
Resistance to multiple chemotherapeutic agents is mainly related to the production of P- glycoproteins, a transmembrane drug efflux pump that is encoded by the multidrug resistance(MDR) gene, MDR I. To investigate whether MDR 1 could be involved in clinical resistance to chemotherapy in adult acute leukemias, We have analyzed prospectively 39 samples from 32 patients(27 at presentation, 5 after relapse) with acute leukemia for the evaluation of overexpression of MDR 1 mRNA and P-glycoprotein by using polymerase chain reactions(PCR) and flow cytometric assay, respectively. The induction rate of first complete remission differed between MDR 1 mRNA-positive and negative groups(37% v 84%, respectively; p=0.02). The survival rate(Kaplan-Meier method) in MDR 1 mRNA negative group was significantly higher than that in MDR 1 mRNA positive group(p=0.02). These results suggest that PCR assay of MDR 1 mRNA is a convenient method to detect expression of MDR 1 gene, and it may be a prognostic factor in the treatment of acute leukemia.
Background The therapeutic outcome for relapsed or refractory adults with acute leukemia is so poor, and it is difficult to expect the long-term disease-free survival in these patients. We evaluated the therapeutic outcome of a salvage chemotherapy consisting of high- or intermediate-dose(HD or ID) cytarabine and a new daunorubicin analogue, idarubicin. Material and method: Twenty one patients with refractory or relapsed acute lymphocytic leukemia(ALL) and 13 patients with acute myeloid leukemia(AML) were treated with a regimen that included idarubicin 12 mg/§³ intravenously daily for 3 days plus HD cytara- bine(3,000 mg/§³ by infusion over 2 hours daily for 3 days) or ID cytarabine(1,000 mg/§³ every 12 hours for six doses). Results: 1) Complete remission(CR) was achieved in thirteen of 34 patients (38%; 10 of relapsed and 3 of refractory): 16 patients(47%) did not respond to the treatment and 5 patients(15%) died during chemotherapy. 2) The median days to the neutrophils over 500/ul was 23 from the initiation of chemotherapy (range 12-39). The significant non-hematologic taxicities were not observed. 3) Two of 13 patients who achieved CR relapsed within 2 months, four relapsed 2 months after CR. Remaining seven patients have been in continuous CR(CCR). 4) For all complete responders, the median CR duration was 6 months, and the projected actuarial disease-free survival rate was 32% at 9 months. For the all patients, the projected overall survival was 15% at 18 months. Conclusion: We found that HD or ID cytarabine and idarubicin can be one of the effective salvage regimens for patients with relapsed acute leukemia. To improve remission rate of refractory cases, the modification of administration and combination therapy with other non-cross resistant drug will be designed.