Children with chronic-phase chronic myeloid leukemia: characteristics and outcomes from the International Registry of Childhood CML
HAEMATOLOGICA, cilt.111, sa.4, ss.1280-1288, 2026 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 111 Sayı: 4
- Basım Tarihi: 2026
- Doi Numarası: 10.3324/haematol.2025.288283
- Dergi Adı: HAEMATOLOGICA
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, BIOSIS, EMBASE, MEDLINE, Directory of Open Access Journals
- Sayfa Sayıları: ss.1280-1288
- Açık Arşiv Koleksiyonu: AVESİS Açık Erişim Koleksiyonu
- Bursa Uludağ Üniversitesi Adresli: Evet
Özet
Five hundred and thirty-five patients (median age 12.2 years; range, 8 months to 18 years; N=303 males, N=232 females) diagnosed with chronic myeloid leukemia (CML) in chronic phase were registered in the International Registry of Childhood CML ( clinicaltrials gov. Identifier: NCT01281735). ). Clinical signs of leukostasis or bleeding were observed in 16.5% and 20% of the patients, respectively. The spleen was palpable in 76% of patients with a median of 9 cm (range, 1-32) below the costal margin. The median leukocyte count was 222x109/L 9/L (interquartile range, 95-353). First-line therapy consisted of imatinib in 482 children. Among them, 40% remained on imatinib with a median follow-up 3.8 years (95% confidence interval [CI]: 3.2-4.3 years); 3.1% died and 3.9% progressed to advanced-phase disease. The overall survival rate at 36 months was 97.4% (95% CI: 95-99%). Progression-free survival rate at 3-years was 97.1% (95% CI: 94.9-99.2%), 91.7% (95% CI:84.5-98.3%) and 72.0% (95% CI: 59-87.9%) in the Eutos Long-Term Survival (ELTS) low-, intermediate-, and high-risk group, respectively (P P log rank <0.001). Pediatric CML is characterized by bulky disease (large splenomegaly, high leukocyte counts) and the ELTS score can identify children with the poorest outcome.