Researchers from St Jude Children’s Research Hospital and researchers from Italy have reported that they have identified a subset of patients with T-cell acute lymphoblastic leukemia (ALL), called ETP-ALL, which connotes an extremely poor prognosis. The details of this study appeared in the February 2009 issue of Lancet Oncology.[1]
T-cell ALL comprises approximately 10-15% ALL diagnoses in children. Historically, Patients with T-cell ALL had a worse prognosis compared with patients with B-cell ALL. However, recent results from the Dana-Farber Cancer Institute have reported a five-year event-free survival rate of 75% for children with T-cell ALL, which is approximately 10% less than for children with B-cell ALL.[2] Treatment in this study consisted of a four- or five-drug induction regimen and consolidation therapy with doxorubicin, vincristine, corticosteroids, mercaptopurine, and asparaginase.
It is important to investigate causes of failure within subgroups of children with T-cell ALL in order to design better treatments. The authors of the current study attempted to determine the causes of failure in the approximately 25% of children with T-cell ALL who succumb to their disease. They looked at cellular characteristics of 139 patients with T-cell ALL treated at St Jude and 100 treated in Italy. They identified 30 patients (13%) who had lymphoblasts with a distinctive gene expression signature called ETP-ALL. Seventy-five percent of children with ETP-ALL had failure of remission induction or relapse at 10 years compared with 10% in children with non-ETP-T-cell ALL treated at St Jude. The remission failure and relapse rates in the Italian cohort were 57% and 14%, respectively.
These authors stated that “ETP-ALL is a distinct, previously unrecognized, pathobiological entity that confers a poor prognosis with the use of standard intensive chemotherapy.”
Comments: This study will allow for the identification of a distinct poor risk group of children with T-cell ALL and should allow for the development of more effective treatment strategies. One strategy that should be immediately considered is early allogeneic stem cell transplantation.
References:
[1] Coustan-Smit E, Mulighan CG, Onciu M, et al. Early T-cell precursor leukaemia: a subtype of very high-risk acute lymphoblastic leukaemia. Lancet Oncology. 2009;10:147-156.
[2] Goldberg JM, Silverman LB, Levy DE, et al. Childhood T-cell acute lymphoblastic leukemia: the Dana-Farber Cancer Institute acute lymphoblastic leukemia consortium experience. Journal of Clinical Oncology. 2003; 21:3616-3622
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