Outcomes of down syndrome-related myeloid leukemia in Turkiye: a multicenter retrospective observational cohort study by the Turkish Pediatric Hematology Association

Children with Down syndrome (DS) have a 150-times increased risk of myeloid leukemia (ML). In DS patients, in addition to DS-ML, common acute myeloid leukemia (AML) subtypes (standard-AML), observed in patients without DS, may also be seen. We evaluated ML types developing among DS cases, along with clinical features, survival, relapse, and mortality rates. The study included DS children aged 6 months to 18 years with newly diagnosed ML. Patients were classified retrospectively into two groups according to WHO guidelines and BFM-AML protocol recommendations as DS-ML and standard AML-DS. Among 53 DS patients, 71.7% (n = 38) had DS-ML and 28.3% (n = 15) had standard AML-DS. The mean age in the DS-ML group was significantly lower than the standard AML-DS group (23 ± 9 vs. 48 ± 51 months, p = 0.033). The 5-year overall survival (OS) and event-free survival (EFS) in the DS-ML group were 84.1%±6.0% and 81.3%±6.4%, respectively. For the standard AML, OS and EFS were 53.3%±12.9% and 45.7%±13.1%, respectively. The DS-ML group had significantly higher OS and EFS than the standard AML-DS group (p = 0.039 and p = 0.025, respectively). In our study, DS-ML and standard AML-DS were evaluated separately, which significantly contributes to the literature on the clinical and laboratory features of both groups, given the limited data on these topics. In the DS-ML group, complete remission, OS, and EFS were significantly higher than in the standard AML-DS. Differentiating DS-ML from standard AML-DS will ensure that patients with these biologically distinct diseases receive treatment of appropriate intensity and increase survival by reducing early mortality and relapse risk.

Authors

Institutions

Publication Details

Journal
Annals of Hematology
Published
2026-09-15
DOI
https://doi.org/10.1007/s00277-026-07245-0
Primary Topic
Acute Myeloid Leukemia Research
Type
article
Field-Weighted Citation Impact
0.00
Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
article

Outcomes of down syndrome-related myeloid leukemia in Turkiye: a multicenter retrospective observational cohort study by the Turkish Pediatric Hematology Association

Ali Fettah, Mehmet Fatih Orhan, Canan Albayrak, Feryal Karahan et al.
Annals of Hematology
Acute Myeloid Leukemia Research
article

Outcomes of down syndrome-related myeloid leukemia in Turkiye: a multicenter retrospective observational cohort study by the Turkish Pediatric Hematology Association

Ali Fettah, Mehmet Fatih Orhan, Canan Albayrak, Feryal Karahan, Suar Çakı Kılıç, Emine Zengin, Ayşenur Bahadır, Elif Güler Kazancı, Neşe Yaralı, Güven Özkaya, Veysiye Hülya Üzel, Yusuf Ziya Aral, Nihal Karadaş, Melike Sezgin Evim, Yeter Düzenli Kar, Hale Ören, Tuba Hilkay Karapınar, Arzu Akyay, Yurday Öncül, Burcu Tufan Taş, Murat Söker, Adalet Meral Güneş, Deniz Yılmaz Karapınar, Ekin Gün, Betül Orhaner
article en

Abstract

Children with Down syndrome (DS) have a 150-times increased risk of myeloid leukemia (ML). In DS patients, in addition to DS-ML, common acute myeloid leukemia (AML) subtypes (standard-AML), observed in patients without DS, may also be seen. We evaluated ML types developing among DS cases, along with clinical features, survival, relapse, and mortality rates. The study included DS children aged 6 months to 18 years with newly diagnosed ML. Patients were classified retrospectively into two groups according to WHO guidelines and BFM-AML protocol recommendations as DS-ML and standard AML-DS. Among 53 DS patients, 71.7% (n = 38) had DS-ML and 28.3% (n = 15) had standard AML-DS. The mean age in the DS-ML group was significantly lower than the standard AML-DS group (23 ± 9 vs. 48 ± 51 months, p = 0.033). The 5-year overall survival (OS) and event-free survival (EFS) in the DS-ML group were 84.1%±6.0% and 81.3%±6.4%, respectively. For the standard AML, OS and EFS were 53.3%±12.9% and 45.7%±13.1%, respectively. The DS-ML group had significantly higher OS and EFS than the standard AML-DS group (p = 0.039 and p = 0.025, respectively). In our study, DS-ML and standard AML-DS were evaluated separately, which significantly contributes to the literature on the clinical and laboratory features of both groups, given the limited data on these topics. In the DS-ML group, complete remission, OS, and EFS were significantly higher than in the standard AML-DS. Differentiating DS-ML from standard AML-DS will ensure that patients with these biologically distinct diseases receive treatment of appropriate intensity and increase survival by reducing early mortality and relapse risk.

Annals of Hematology
Sakarya University (TR), Dicle University (TR), Bursa Uludağ Üni̇versi̇tesi̇ (TR), Ankara University (TR), Karadeniz Technical University (TR), Bilkent University (TR), University of Health Science (KH), Bursa Technical University (TR), Dokuz Eylül University (TR), Inonu University (TR), Ondokuz Mayıs University (TR), Ege University (TR), Dr. Behçet Uz Çocuk Hastalıkları Hastanesi (TR), Memorial Ankara Hospital (TR), Ümraniye Eğitim ve Araştırma Hastanesi (TR), Başkent University Hospital (TR), Sağlık Bilimleri Üniversitesi (TR), Bursa Yuksek Ihtisas Egitim Ve Arastirma Hastanesi (TR), Mersin Üniversitesi (TR), Kocaeli Üniversitesi (TR), Marmara University (TR), Adnan Menderes University (TR)
Good health and well-being
Openalex Percentile: Top 11%
Acute Myeloid Leukemia Research
DOIOpen Access PDFOpenAlex
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.