BACKGROUND: The presenting features and treatment outcome of 120 patients with Down syndrome (DS) and childhood acute lymphoblastic leukemia (ALL) were compared with 6237 non-DS patients treated in the same years. METHODS: We reviewed the database of 6 consecutive Italian Association of Pediatric Hematology and Oncology (AIEOP)-ALL trials conducted between 1982 and 2004. Features of DS patients were compared with those of non-DS patients. RESULTS: The 120 DS patients (1.9%) were more often girls (P = .027), aged > or = 10 years (P = .014), and high risk according to National Cancer Institute (NCI) criteria (P = .045). The distribution of white blood cell count did not differ (P = .32). DS patients belonged less frequently to the current high-risk group (P = .017). In all but 1 case they demonstrated B-cell precursor (BCP) immunophenotype (P < or = .001). TEL/AML1 molecular fusion transcript was found in only 1 of 44 (2.2%) tested patients. Induction death occurred more often in DS patients (4.2%, P = .009), but not failure to achieve remission. Leukemia relapse occurred in 31.6% of DS patients (vs 23.5%; P = .003), usually in the marrow. Remission death was more frequent in DS patients (4.2%, P = .03). Ten-year event-free survival and survival were significantly worse compared with non-DS patients (P < 0.001). DS patients diagnosed since 1995 had a better outcome (P = .06) than those diagnosed in previous years, but still had worse outcomes than non-DS patients (P = .04). Event-free survival of DS patients at NCI standard risk was lower than that of non-DS patients (P = .006). CONCLUSIONS: Presenting features of childhood ALL in DS differ from those in non-DS patients. They are almost invariably characterized by BCP phenotype, and are often TEL/AML1 negative. Treatment results, although not as good as for non-DS patients, improved progressively, with modern therapy and support allowing 75% to survive. (c) 2008 American Cancer Society
BACKGROUND: The presenting features and treatment outcome of 120 patients with Down syndrome (DS) and childhood acute lymphoblastic leukemia (ALL) were compared with 6237 non-DS patients treated in the same years. METHODS: We reviewed the database of 6 consecutive Italian Association of Pediatric Hematology and Oncology (AIEOP)-ALL trials conducted between 1982 and 2004. Features of DS patients were compared with those of non-DS patients. RESULTS: The 120 DS patients (1.9%) were more often girls (P = .027), aged > or = 10 years (P = .014), and high risk according to National Cancer Institute (NCI) criteria (P = .045). The distribution of white blood cell count did not differ (P = .32). DS patients belonged less frequently to the current high-risk group (P = .017). In all but 1 case they demonstrated B-cell precursor (BCP) immunophenotype (P < or = .001). TEL/AML1 molecular fusion transcript was found in only 1 of 44 (2.2%) tested patients. Induction death occurred more often in DS patients (4.2%, P = .009), but not failure to achieve remission. Leukemia relapse occurred in 31.6% of DS patients (vs 23.5%; P = .003), usually in the marrow. Remission death was more frequent in DS patients (4.2%, P = .03). Ten-year event-free survival and survival were significantly worse compared with non-DS patients (P < 0.001). DS patients diagnosed since 1995 had a better outcome (P = .06) than those diagnosed in previous years, but still had worse outcomes than non-DS patients (P = .04). Event-free survival of DS patients at NCI standard risk was lower than that of non-DS patients (P = .006). CONCLUSIONS: Presenting features of childhood ALL in DS differ from those in non-DS patients. They are almost invariably characterized by BCP phenotype, and are often TEL/AML1 negative. Treatment results, although not as good as for non-DS patients, improved progressively, with modern therapy and support allowing 75% to survive. (c) 2008 American Cancer Society
Authors: Kelly W Maloney; William L Carroll; Andrew J Carroll; Meenakshi Devidas; Michael J Borowitz; Paul L Martin; Jeanette Pullen; James A Whitlock; Cheryl L Willman; Naomi J Winick; Bruce M Camitta; Stephen P Hunger Journal: Blood Date: 2010-05-04 Impact factor: 22.113
Authors: Elizabeth G Salazar; Yimei Li; Brian T Fisher; Susan R Rheingold; Julie Fitzgerald; Alix E Seif; Yuan-Shung Huang; Rochelle Bagatell; Richard Aplenc Journal: Br J Haematol Date: 2016-05-10 Impact factor: 6.998
Authors: Yousif Matloub; Karen R Rabin; Lingyun Ji; Meenakshi Devidas; Johann Hitzler; Xinxin Xu; Bruce C Bostrom; Linda C Stork; Naomi Winick; Julie M Gastier-Foster; Nyla A Heerema; Eileen Stonerock; William L Carroll; Stephen P Hunger; Paul S Gaynon Journal: Blood Adv Date: 2019-06-11
Authors: Alix E Seif; Brian T Fisher; Yimei Li; Kari Torp; Douglas P Rheam; Yuan-Shung V Huang; Tracey Harris; Ami Shah; Matthew Hall; Evan S Fieldston; Marko Kavcic; Marijana Vujkovic; L Charles Bailey; Leslie S Kersun; Anne F Reilly; Susan R Rheingold; Dana M Walker; Richard Aplenc Journal: Pediatr Blood Cancer Date: 2013-11-19 Impact factor: 3.167