Biological and clinical features of trisomy 21 in adult patients with acute myeloid leukemia

Clin Lymphoma Myeloma Leuk. 2013 Sep;13 Suppl 2(0 2):S276-81. doi: 10.1016/j.clml.2013.05.020. Epub 2013 Aug 19.

Abstract

Introduction: Trisomy 21 is frequently noted in patients with AML. In adults, +21 has traditionally been considered an intermediate-risk cytogenetic aberration.

Patients and methods: We analyzed 90 patients with newly diagnosed AML harboring +21. Four cytogenetic subgroups were defined based on associated cytogenetic abnormalities: +21 alone, +21 with favorable, +21 with intermediate, and +21 with unfavorable cytogenetics.

Results: Fifty-four percent of patients with +21 AML achieved a complete remission (CR) or CR with incomplete platelet recovery (CRp) after induction therapy with a trend toward improved CR/CRp rates in patients with +21 alone/+21 with favorable cytogenetics compared with patients with +21 with intermediate/+21 with unfavorable cytogenetics (76% vs. 50%; P = .057). Time to progression (TTP) was 12 months (range, 5-19) and overall survival (OS) was 9 months (range, 7-11) for the entire group. TTP was longer for patients with +21 alone (not reached) or with +21 with favorable cytogenetics (101 months) compared with those with +21 with intermediate cytogenetics (2 months) or +21 with unfavorable cytogenetics (11 months) (P = .02). Similarly, OS was improved in patients with +21 with favorable cytogenetics (not reached) or +21 alone (107 months), compared with +21 with unfavorable cytogenetics (9 months) or +21 with intermediate cytogenetics (8 months) (P < .001). The differences in TTP and OS were maintained on multivariate analysis (P = .04 and P = .001; respectively).

Conclusion: Isolated +21 hitherto classified as intermediate-risk cytogenetics might actually behave as a favorable-risk cytogenetics in adult AML patients.

Keywords: AML; Cytogenetic; Down syndrome; Prognosis; trisomy 21.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Cytogenetics
  • Disease-Free Survival
  • Down Syndrome / genetics
  • Down Syndrome / pathology*
  • Female
  • Humans
  • Karyotyping
  • Leukemia, Myeloid, Acute / genetics*
  • Leukemia, Myeloid, Acute / pathology*
  • Male
  • Middle Aged
  • Prognosis
  • Treatment Outcome
  • Young Adult