A patient with Down's syndrome (Trisomy 21) is at increased risk for acute myeloid leukemia (AML) and acute lymphoblastic leukemia (ALL). ALL in a patient with Down's syndrome differs in some ways from that seen in other patients.
Patients at risk:
(1) female gender
(2) age > 10 years of age
Features of the leukemia:
(1) often high risk by the NCI criteria
(2) usually show the B-cell precursor (BCP) immunophenotype
(3) usually TEL/AML1 (a molecular fusion transcript) negative
Risks compared to patients without Down's syndrome:
(1) higher rate of induction deaths (4%)
(2) higher rate of leukemia relapse (32%)
(3) higher rate of remission deaths (4%)
(4) lower overall and event free survival
The event free survival at 10 years for all Down's syndrome patients is around 61%. The overall survival at 10 years is around 76%.
