Outcome of children with advanced T-cell lymphoid malignancy treated according to a modified POG protocol

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Children with acute lymphoblastic leukemia of T-cell origin, and T-lineage advanced stage lymphoblastic lymphoma are at a high risk of treatment failure. The Pediatric Oncology Group (POG) adopted a phenotype-specific treatment approach for patients with these disease entities.1 Based on their approach we have used a modification of their protocol to treat patients with advanced stage T-cell lymphoid malignancies at KFSH&RC. The modifications of the POG protocol were made to eliminate epipodophyllotoxin use, and replaced the teniposide/cytarabine (Ara-C) sequences with intermediate dose methotrexate (IDMTX) (1gm/m2) and high dose Ara-C (2gm/m2 q 12h X 4) in consolidation, and with IDMTX, Ara-C (300mg/m2 daily X 2) and L-asparaginase (10,000U/m2/week X 3) in the maintenance cycles.
Results: Between January '96 and December '98, seventeen patients were enrolled for treatment on this protocol. Three of these patients had stage III or IV NHL. There were eleven boys and the median age at presentation was 7 years (range 2.8-14). Seven patients had wbc counts ≥50 × 109/L, and four had leukemic blasts in the CSF. All patients achieved complete remission. The treating physician switched two patients to a different treatment regimen during maintenance chemotherapy, and a third patient underwent allogeneic bone marrow transplantation in first remission. Fourteen patients continued on therapy according to this protocol, and are the subject of this analysis. At the time of this evaluation (January 2000) five patients had relapsed. All relapses occurred during maintenance chemotherapy. By Kaplan-Meier analysis, the projected relapse free survival at 2 years, for all the patients, is 48%. For the 11 patients with T-cell ALL it is 27%. Several patients continue on treatment according to this protocol, and remain at risk for relapse.
Conclusion: This treatment regimen, based on the POG protocols for advanced T-cell lymphoid malignancies, is not effective therapy for our patient population. This could either be a result of the modifications made in the protocol, or possibly due to a more aggressive phenotype in our patients.


This work was published before the author joined Aga Khan University

Publication (Name of Journal)

Journal of Pediatric Hematology/Oncology