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Childhood T-Cell Acute Lymphoblastic Leukemia: The Dana-Farber Cancer Institute Acute Lymphoblastic Leukemia Consortium Experience

John M. Goldberg, Lewis B. Silverman, Donna E. Levy, Virginia Kimball Dalton, Richard D. Gelber, Leslie Lehmann, Harvey J. Cohen, Stephen E. Sallan, Barbara L. Asselin

From the Department of Pediatric Oncology and the Department of Biostatistical Science, Dana-Farber Cancer Institute; the Division of Hematology/Oncology, Children’s Hospital and Department of Pediatrics, Harvard Medical School, Boston, MA; the Department of Pediatrics, Stanford University School of Medicine, Stanford, CA; the Department of Pediatrics, Division of Hematology/Oncology, University of Rochester Medical Center, Rochester, NY.

Address reprint requests to Barbara Asselin, MD, 601 Elmwood Ave, Box 777, Rochester, NY 14642; e-mail: barbara_asselin{at}urmc.rochester.edu.

Purpose: T-cell acute lymphoblastic leukemia (T-ALL) accounts for 10% to 15% of newly diagnosed cases of childhood acute lymphoblastic leukemia (ALL). Historically, T-ALL patients have had a worse prognosis than other ALL patients.

Patients and Methods: We reviewed the outcomes of 125 patients with T-ALL treated on Dana-Farber Cancer Institute (DFCI) ALL Consortium trials between 1981 and 1995. Therapy included four- or five-agent remission induction; consolidation therapy with doxorubicin, vincristine, corticosteroid, mercaptopurine, and weekly high-dose asparaginase; and cranial radiation. T-ALL patients were treated the same as high-risk B-progenitor ALL patients. Fifteen patients with T-cell lymphoblastic lymphoma were also treated with the same high-risk regimen between 1981 and 2000.

Results: The 5-year event-free survival (EFS) rate for T-ALL patients was 75% ± 4%. Fourteen of 15 patients with T-cell lymphoblastic lymphoma were long-term survivors. There was no significant difference in EFS comparing patients with T-ALL and B-progenitor ALL (P = .56), although T-ALL patients had significantly higher rates of induction failure (P < .0001), and central nervous system (CNS) relapse (P = .02). The median time to relapse in T-ALL patients was 1.2 years versus 2.5 years in B-progenitor ALL patients (P = .001). There were no pretreatment characteristics associated with worse prognosis in patients with T-ALL.

Conclusion: T-ALL patients fared as well as B-progenitor patients on DFCI ALL Consortium protocols. Patients with T-ALL remain at increased risk for induction failure, early relapse, and isolated CNS relapse. Future studies should focus on the identification of and treatment for T-ALL patients at high risk for treatment failure.

Supported in part by grants from the National Institutes of Health (CA 68484 and 06516).

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