Document Detail

Acute lymphoblastic leukemia and Down syndrome: presenting features and treatment outcome in the experience of the Italian Association of Pediatric Hematology and Oncology (AIEOP).
MedLine Citation:
PMID:  18521927     Owner:  NLM     Status:  MEDLINE    
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.
Maurizio Arico; Ottavio Ziino; Maria Grazia Valsecchi; Giovanni Cazzaniga; Carlo Baronci; Chiara Messina; Andrea Pession; Nicola Santoro; Giuseppe Basso; Valentino Conter;
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Publication Detail:
Type:  Comparative Study; Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Cancer     Volume:  113     ISSN:  0008-543X     ISO Abbreviation:  Cancer     Publication Date:  2008 Aug 
Date Detail:
Created Date:  2008-07-28     Completed Date:  2008-09-10     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0374236     Medline TA:  Cancer     Country:  United States    
Other Details:
Languages:  eng     Pagination:  515-21     Citation Subset:  AIM; IM    
Copyright Information:
(c) 2008 American Cancer Society
Pediatric Hematology and Oncology, Children Hospital "A.O.U. Meyer," Florence, Italy.
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MeSH Terms
Child, Preschool
Clinical Trials as Topic
Down Syndrome / complications,  diagnosis*,  mortality,  therapy*
Italy / epidemiology
Precursor Cell Lymphoblastic Leukemia-Lymphoma / complications,  diagnosis*,  mortality,  therapy*
Retrospective Studies
Survival Analysis
Treatment Outcome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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