1. TEL/AML1 Rearrangement Is Rare in Children with Acute Lymphoblastic Leukemia (ALL) and Down Syndrome (DS). Presenting Features and Treatment Outcome in the Experience of the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP)
- Author
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Matteo Luciani, Concetta Micalizzi, Chiara Messina, Giuseppe Masera, Anna Maria Testi, Daniela Silvestri, Andrea Biondi, Andrea Pession, Giovanni Cazzaniga, Luca Lo Nigro, Maurizio Aricò, Ottavio Ziino, Maria Grazia Valsecchi, Franco Locatelli, Valentino Conter, Carmelo Rizzari, Giuseppe Basso, Nicola Santoro, Fiorina Casale, Elena Barisone, and Rosanna Parasole
- Subjects
Down syndrome ,medicine.medical_specialty ,Pediatrics ,business.industry ,Lymphoblastic Leukemia ,Immunology ,Treatment outcome ,Complete remission ,Cell Biology ,Hematology ,medicine.disease ,Biochemistry ,Gastroenterology ,Immunophenotyping ,medicine.anatomical_structure ,Leukemia relapse ,Internal medicine ,medicine ,Tel aml1 ,Bone marrow ,business - Abstract
We compared the clinical and biologic features and treatment results of patients with DS-ALL (n=120, 1.9%), compared to those without DS (n=6237), enrolled in the AIEOP trials between 1988 and October 2004. DS patients had significantly more often the following characteristics: female gender (55.0% vs 44.9%, p=0.027), age 10 years or older (28.3 vs 17.7%, p=0.014), high risk by NCI criteria (41.7 vs. 33%, p=0.045); on the contrary significantly less often they belonged to high-risk group according to current stratification (10.8 vs. 20.3%, p=0.017) or had T-lineage immunophenotype (0.8 vs 11.8%, p=100K/mm3: 6.6 vs. 10.8%). TEL/AML1 rearrangement was found in only 1 of 44 tested (2.2%). Of the 120 DS patients, 5 died during induction (4.2 vs 1.2% in non-DS), 1 was resistant (0.8 vs 1.4%). Leukemia relapse occurred in 31.6% of DS patients, compared with 24.9% of non-DS: bone marrow 22.5 vs 15.5%, CNS isolated 4.2 vs 3.6%, testis 0.8 vs 1.6%, BM combined 3.3 vs. 3.6%, other site 0.8 vs 0.6%. Death in complete remission occurred in 4.2 vs 2.0%. No second malignancy was reported. Overall, 59.2% of DS patients remained in first remission, compared with 70.2% of non-DS patients. This translated into a probability of EFS (SE) at 10 years of 56.2% (4.8) versus 67.7% (0.6) and a survival of 60.8% (5.0) versus 77.2% (0.6). 57 DS patients were diagnosed
- Published
- 2007