Treatment results in children with myeloid leukemia of Down syndrome in Saudi Arabia: A multicenter SAPHOS leukemia group study. (July 2017)

• Record Type: Journal Article
• Authors: Jastaniah, Wasil; Alsultan, Abdulrahman; Al Daama, Saad; Ballourah, Walid; Bayoumy, Mohammad; Al-Anzi, Faisal; Al Shareef, Omar; Abrar, Mohammed Burhan; Al Sudairy, Reem; Al Ghemlas, Ibrahim
• Is Part Of: Leukemia research. Volume 58(2017:Jul.)
• Page Start: 48
• Page End: 54
• Publication Date: 2017-07
• Subjects: Myeloid leukemia -- Down syndrome -- Child -- Prognosis -- Survival; Leukemia -- Periodicals; Leukemia -- Periodicals; Leucémie -- Périodiques; Leukemia; Periodicals; Electronic journals; Electronic journals; 616.9941905
• DOI: 10.1016/j.leukres.2017.04.004
• Languages: English
• ISSNs: 0145-2126

Abstract

Highlights: The incidence of myeloid leukemia of DS (ML-DS) in Saudi Arabia was 48 per 100, 000. Clinical and laboratory characteristics of ML-DS were comparable to other reports. Treatment outcomes varied based on associated cytogenetic abnormalities. Optimizing risk stratification and treatment intensity is warranted in ML-DS. Abstract: Despite the high incidence of Down syndrome (DS) in Arab countires, the incidence and outcomes of myeloid leukemia of DS (ML-DS) have not been studied. We evaluated 206 pediatric acute myeloid leukemia (AML) patients diagnosed between 2005 and 2012 and identified 31 (15%) ML-DS. The incidence of ML-DS was 48 per 100, 000 compared to 0.6 per 100, 000 for AML in non-DS children. Thus, patients with DS had 80-fold increased risk of ML-DS compared to AML in non-DS children. The median age at diagnosis was 1.8 years, male/female ratio was 1.2, majority (84%) of patients had FAB-M7 subtype, and the cytogenetic abnormalities were normal karyotype (constitutional trisomy 21) in 48%, additional trisomy in 23%, and other aberrations in 29%. Complete remission, cumulative incidences of relapse (CIR), toxic-death, and 5-year event-free survival (EFS) rates were 96.8%, 19.4%, 13.1%, and 67.7 ± 8.4%; respectively. In the present study, multivariate analysis revealed favorable outcome (5-year EFS 86.7 ± 8.8%) for patients with normal karyotype. The incidence and clinical characteristics of ML-DS in Saudi patients were comparable to other reports. However, there is a need to optimize risk stratification and treatment intensity to reduce CIR and toxic death rates to further improve outcomes of patients with ML-DS.