Characteristics of patients with TEL-AML1-positive acute lymphoblastic leukemia with single or multiple fusions

Pediatr Blood Cancer. 2007 May;48(5):510-4. doi: 10.1002/pbc.20911.

Abstract

Background: The TEL-AML1 fusion in precursor-B ALL is generated by a cryptic 12;21 translocation that is detectable by fluorescence in situ hybridization (FISH). It is generally considered a favorable prognostic indicator. Some TEL-AML1+ ALL patients present at diagnosis with extra copies of the fusion, enumerated by FISH. The aim of the study was to determine whether additional copies of TEL-AML1 have clinical significance.

Procedure: Charts of all TEL-AML1+ ALL patients at the UM and Children's Hospitals and Clinics of Minnesota between 1996 and 2004 were reviewed.

Results: Eight patients (7 males/1 female, mean age 46 months) with two or more TEL-AML1 fusion signals and 24 with single TEL-AML1 fusion signals (18 males/6 females, mean age 52 months) were identified. There was no statistically significant difference in age or gender between the two groups. Patients with double TEL-AML1+ had a higher frequency of myeloid markers CD13 (P = 0.04) or CD33 (P = 0.003) than single TEL-AML1+ patients. Single TEL-AML1+ patients had higher WBC (P = 0.04) than double TEL-AML1+ patients. A trend toward slower therapy response was seen in double TEL-AML1+ patients versus single, (1 of 7 [14%] <5% marrow blasts on Day 7 vs. 13 of 23 [56%], P = 0.09). Double TEL-AML1+ patients had a higher relapse rate (P = 0.09) than single TEL-AML1+ patients.

Conclusions: Utilizing FISH to distinguish subgroups of TEL-AML1 fusion patients may have important prognostic implications. The presence of an extra fusion may portend poorer prognosis. A larger and longer-term follow-up study will be required to verify the possible clinical significance of the presence of multiple TEL-AML1 fusions.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antigens, CD / analysis
  • Antigens, Differentiation, Myelomonocytic / analysis
  • CD13 Antigens / analysis
  • Child
  • Child, Preschool
  • Chromosomes, Human, 21-22 and Y
  • Chromosomes, Human, 6-12 and X
  • Core Binding Factor Alpha 2 Subunit / genetics*
  • ETS Translocation Variant 6 Protein
  • Female
  • Humans
  • In Situ Hybridization, Fluorescence
  • Infant
  • Leukocyte Count
  • Male
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics*
  • Prognosis
  • Proto-Oncogene Proteins c-ets / genetics*
  • Repressor Proteins / genetics*
  • Retrospective Studies
  • Sialic Acid Binding Ig-like Lectin 3
  • Translocation, Genetic

Substances

  • Antigens, CD
  • Antigens, Differentiation, Myelomonocytic
  • CD33 protein, human
  • Core Binding Factor Alpha 2 Subunit
  • Proto-Oncogene Proteins c-ets
  • RUNX1 protein, human
  • Repressor Proteins
  • Sialic Acid Binding Ig-like Lectin 3
  • CD13 Antigens