TEL/AML-1 dimerizes and is associated with a favorable outcome in childhood acute lymphoblastic leukemia

McLean TW, Ringold S, Neuberg D, Stegmaier K, Tantravahi R, Ritz J, Koeffler HP, Takeuchi S, Janssen JW, Seriu T, et al. TEL/AML-1 dimerizes and is associated with a favorable outcome in childhood acute lymphoblastic leukemia. Blood. 1996;88:4252–8.

NOTES

McLean, T WRingold, SNeuberg, DStegmaier, KTantravahi, RRitz, JKoeffler, H PTakeuchi, SJanssen, J WSeriu, TBartram, C RSallan, S EGilliland, D GGolub, T RengCA 57261/CA/NCI NIH HHS/Research Support, Non-U.S. Gov'tResearch Support, U.S. Gov't, P.H.S.Blood. 1996 Dec 1;88(11):4252-8.

Abstract

Polymerase chain reaction-based screening of childhood acute lymphoblastic leukemia (ALL) samples showed that a TEL/AML1 fusion transcript was detected in 27% of all cases, representing the most common known gene rearrangement in childhood cancer. The TEL/AML1 fusion results from a t(12;21)(p13;q22) chromosomal translocation, but was undetectable at the routine cytogenetic level. TEL/AML1-positive patients had exclusively B-lineage ALL, and most patients were between the ages of 2 and 9 years at diagnosis. Only 3/89 (3.4%) adult ALL patients were TEL/AML1-positive. Most importantly, TEL/AML1-positive children had a significantly lower rate of relapse compared with TEL/AML1-negative patients (0/22 v 16/54, P = .004). Co-immunoprecipitation experiments demonstrated that TEL/AML-1 formed homodimers in vitro, and heterodimerized with the normal TEL protein when the two proteins were expressed together. The elucidation of the precise mechanism of transformation by TEL/AML1 and the role of TEL/AML1 testing in the treatment of childhood ALL will require additional studies.
Last updated on 02/17/2021