Outcome of Treatment in Children with Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia

Chung, Nak Gyn; Lee, Soon Ju; Kim, Sun Young; Jang, Pil Sang; Cho, Bin; Jeong, Dae Chul; Kim, Hack Ki

Abstract

PURPOSE
The prognosis of Philadelphia chromosome-positive acute lymphoblastic leukemiaacute lymphoblastic leukemia with Philadelphia chromosome (Ph ALL) is generally poor and reports from large studies are scarce. We evaluated the clinical characteristics and the efficacy of allogeneic bone marrow transplantation (Allo-BMT) and chemotherapy for children with this type of leukemia. METHODS: Fifteen children diagnosed with Ph ALL among 278 childhood acute lymphoblastic leukemia patients in our St. Mary's hospital from February 1997 to July 2002 were retrospectively reviewed. RESULTS: Of 278 eligible cases, 15 (5.4%) had a Philadelphia chromosome. There were 12 males and 3 females with a median age 9 years (range, 4~13). Initial white blood cell counts ranged from 6.3~446.0 109/L (median, 87.2 109/L). Immunophenotyping studies indicated B-lymphoid phenotype in 13, myeloid/B-lymphoid biphenotype in 2. Of 15 Ph ALL children, 12 patients entered remission after first induction therapy and remained other 3 cases finally had delayed remission. Among evaluable 14 patients, seven received Allo-BMT from HLA-identical sibling or unrelated donor and three of these patients died following recurrence and severe graft versus host disease. Only three patients are alive recently and of them two patients have durable disease free survival more than 24 months. All seven patients who treated with only chemotherapy only died of disease recurrence. CONCLUSION: Unlike the usual type of ALL, Ph ALL is associated with extremely poor prognosis and short remission duration. Therefore, Allo-BMT in first remission was the only treatment modality to cure children with Ph ALL in this study. However, even with Allo-BMT, the relapse rate is quite high. Thus, further study is needed to identify the risk factors affecting treatment outcome and to develop new treatment strategy to overcome this factors.