TY - JOUR
T1 - Acute lymphoblastic leukemia and Down syndrome
T2 - Presenting features and treatment outcome in the experience of the Italian Association of Pediatric Hematology and Oncology (AIEOP)
AU - Arico, Maurizio
AU - Ziino, Ottavio
AU - Valsecchi, Maria Grazia
AU - Cazzaniga, Giovanni
AU - Baronci, Carlo
AU - Messina, Chiara
AU - Pession, Andrea
AU - Santoro, Nicola
AU - Basso, Giuseppe
AU - Conter, Valentino
PY - 2008/8/1
Y1 - 2008/8/1
N2 - 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 ≥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 ≤ .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.
AB - 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 ≥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 ≤ .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.
KW - BFM chemotherapy
KW - Childhood acute lymphoblastic leukemia
KW - DNA index
KW - Down syndrome
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U2 - 10.1002/cncr.23587
DO - 10.1002/cncr.23587
M3 - Article
C2 - 18521927
AN - SCOPUS:49049105472
VL - 113
SP - 515
EP - 521
JO - Cancer
JF - Cancer
SN - 0008-543X
IS - 3
ER -