소아 필라델피아 양성 급성림프구성백혈병의 관해 후 치료

소아 필라델피아 양성 급성림프구성백혈병의 관해 후 치료

Post remission treatment of pediatric Philadelphia chromosome-positive acute lymphoblastic leukemia

(구연):
Release Date : 2017. 10. 26(목)
Seong koo Kim , Yu-Mi Park, NaYeong Lee, Juae Shin, Jae Wook Lee, Pil-Sang Jang, Nack-Gyun Chung , Bin Cho
Catholic University College of Medicine Department of Pediatrics1
김성구, 박유미, 이나영, 신주애, 이재욱, 장필상, 정낙균 , 조빈
가톨릭대학교 의과대학 소아청소년과1

Abstract

Ph+ALL is rare in children, comprising about 3% of ALL patients. Although the TKI has led to improved outcome, Ph+ALL continues to show inferior survival compared to other ALL subgroups. We analyzed 35 Ph+ALL patients treated at The Catholic University of Korea from Aug.2007 to Dec.2016, with the aim of comparing chemotherapy only(N=4)versus allo- HSCT (N=31) as methods of post-remission therapy. All patients started TKI therapy after remission induction as shown in the Children’s Oncology Group(COG)AALL0031 trial. Median follow-up duration was 49 months(range7.9~120.9). All 4 patients in the chemotherapy group relapsed, 1patient with central nervous system (CNS)relapse during maintenance chemotherapy, and 3 with molecular-relapse after treatment completion. Except for 1patient who died of sepsis during reinduction, the remaining patients received HSCT, of whom 2survive disease-free. Of the 31patients who received allo-HSCT in first complete remission(CR), 5relapsed post-transplant, of whom 2survive disease-free after a second HSCT. Four patients died of sepsis(N=2 ) and pulmonary hemorrhage(N=2) post-HSCT in remission. The 3-year EFS and OS in the chemotherapy and HSCT groups were 25±21.7%versus 75±8.1%( P=0.029), and 75±21.7%versus 85.3±6.8%( P=0.642) respectively. In the overall cohort of 35patients, none of the factors studied had a significant impact on EFS. Although comparing groups with disparate patient numbers, we found a significantly lower EFS in patients treated with chemotherapy+TKI only compared with those who received allo-HSCT in first CR. Defining risk factors for relapse may aid in further improving the outcome of this rare ALL subtype.

Keywords: Philadelphia, Acute lymphoblastic leukemia, HSCT