Myeloid leukaemia in children with Down syndrome: report of the registry-based French experience between 1990 and 2003.
OBJECTIVE
To determine the epidemiology of myeloid leukaemia (ML) in children with Down syndrome (DS) and the efficacy of two approaches, low-dose cytarabine-based regimen (LDC) and standard-dose intensive chemotherapy (SD).


METHODS
All children with Down syndrome aged from 2 months to 15 years with ML/myelodysplasia registered in the French registry between January 1990 and December 2003 were included.


RESULTS
Forty-four patients were included. The median age was 1.75 years. The French-America-British subtypes were as follows: M7: 24, M0: 6, M2: 5, M6: 2. Forty-three patients were treated with curative prospect, 20 patients with LDC regimen and 22 according to SD protocols, 1 was given the LDC regimen plus autologous stem-cell transplantation. The event-free survival (EFS) and overall survival (OS) at 5 years were 64.4% and 76.8%. At 5 years, OS in LDC and SD groups were 65% and 85.9% (P = 0.08). EFS were 45% and 80.3% respectively (P < 0.01).


CONCLUSIONS
Children with DS can adequately tolerate SD chemotherapy with a significant superiority of EFS relative to LDC. We suggest that higher levels of cure can be obtained in DS-ML with SD chemotherapy including cytarabine and anthracyclines.
Tandonnet
Julie
J
Bordeaux University and Bordeaux University Hospital, Pediatric Oncology and Hematology Unit, 33076 Bordeaux, France.
Clavel
Jacqueline
J
Baruchel
André
A
Nacka
Fabienne
F
Pérel
Yves
Y
D000970:Antineoplastic Agents / Q000627:therapeutic use*; D002675:Child, Preschool; D003561:Cytarabine / Q000008:administration & dosage / Q000009:adverse effects; D018572:Disease-Free Survival; D004305:Dose-Response Relationship, Drug; D004314:Down Syndrome / Q000150:complications* / Q000188:drug therapy; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D007223:Infant; D007951:Leukemia, Myeloid / Q000150:complications* / Q000188:drug therapy* / Q000453:epidemiology*; D008297:Male; D012042:Registries; D016896:Treatment Outcome
D000970:Antineoplastic Agents; D003561:Cytarabine