Korean J Hematol 2001; 36(1):
Published online March 31, 2001
© The Korean Society of Hematology
소아기 급성 림프구성 백혈병에서 E2A-PBX1 검색에 의한 미세잔존 백혈병의 의의 및 지연강화요법의 효과
서종진, 김은정, 박준은, 서을주, 박찬정, 지현숙, 문형남, 김태형
울산대학교 의과대학 서울중앙병원 소아과,
울산대학교 의과대학 서울중앙병원 임상병리과,
아산생명과학연구소,
단국대학교 의과대학 소아과학교실
The Implications of E2A-PBX1 Positivity and Effect of Delayed Intensification Chemotherapy in t(1;19)-positive Childhood Acute Lymphoblastic Leukemia
Purpose: We studied the E2A-PBX1 positivity in t(1;19)-positive childhood acute lymphoblastic leukemia (ALL) patients during chemotherapy and follow-up period to evaluate the clinical implications of minimal residual disease (MRD) and the
effect
of delayed intensification chemotherapy on long-term survival.
Methods: Fifty-six bone marrow or peripheral blood samples collected retrospectively or prospectively before or during chemotherapy from 14 childhood ALL patients who had t(1;19) or E2A-PBX1 transcript at initial diagnosis were studied for
the
presence of E2A-PBX1 by RT-PCR. All patients received delayed intensification chemotherapy regardless of standard prognostic grouping for childhood ALL to evaluate its effect on the improvement of long-term survival.
Results: There were 11 t(1;19)-positive cases documented by karyotyping and 3 E2A-PBX1 transcript-positive cases amplified by PCR from the initial bone marrow samples. There were 11 cases of FAB L1 and 3 cases of L2. Immunophenotypic
classification revealed 10 cases of group Ⅴ, 2 cases of group Ⅳ, and 1 case of group Ⅲ. Among 11 cases with documented karyotype, 9 cases (81.8%) had der(19)t(1;19) and the other 2 had balanced t(1;19). Fifty-six samples collected at 2 to 7
different
time points of 14 patients revealed 4 cases of MRD immediately after completion of induction chemotherapy despite hematologic complete remission. Three of these cases relapsed eventually, and 1 was lost to follow-up. Among 12 cases who received
adequate
delayed intensificiation chemotherapy, 10 are alive in complete remission.
Conclusion: MRD detection by RT-PCR amplification of E2A-PBX1 transcript of induction chemotherapy was most informative for the prediction of long-term survival and relapse. The presence of MRD after completion of induction chemotherapy
conferred
poor prognosis. The addition of delayed intensification chemotherapy to standard chemotherapy regimen could abolish the adverse effect of t(1;19) in childhood ALL patients.
Keywords E2A-PBX1; t(1;19); Minimal residual disease; Childhood ALL; Chemotherapy;
PDF
Standard view
Export citation
Share Article
Korean J Hematol 2001; 36(1): 60-70
Published online March 31, 2001
Copyright © The Korean Society of Hematology.
소아기 급성 림프구성 백혈병에서 E2A-PBX1 검색에 의한 미세잔존 백혈병의 의의 및 지연강화요법의 효과
서종진, 김은정, 박준은, 서을주, 박찬정, 지현숙, 문형남, 김태형
울산대학교 의과대학 서울중앙병원 소아과,
울산대학교 의과대학 서울중앙병원 임상병리과,
아산생명과학연구소,
단국대학교 의과대학 소아과학교실
The Implications of E2A-PBX1 Positivity and Effect of Delayed Intensification Chemotherapy in t(1;19)-positive Childhood Acute Lymphoblastic Leukemia
Jong Jin Seo, Eun Jung Kim, Jun Eun Park, Eul Ju Seo, Chan Jeoung Park, Hyun Sook Chi, Hyung Nam Moon, Tae Hyoung Kim
Department of Pediatrics and Clinical Pathology, Asan Medical Center, Ulsan University College of Medicine, Asan Institute for Life Sciences, Seoul
Department of Pediatrics, Don Kook University College of Medicine, Chonan, Korea
Abstract
Purpose: We studied the E2A-PBX1 positivity in t(1;19)-positive childhood acute lymphoblastic leukemia (ALL) patients during chemotherapy and follow-up period to evaluate the clinical implications of minimal residual disease (MRD) and the
effect
of delayed intensification chemotherapy on long-term survival.
Methods: Fifty-six bone marrow or peripheral blood samples collected retrospectively or prospectively before or during chemotherapy from 14 childhood ALL patients who had t(1;19) or E2A-PBX1 transcript at initial diagnosis were studied for
the
presence of E2A-PBX1 by RT-PCR. All patients received delayed intensification chemotherapy regardless of standard prognostic grouping for childhood ALL to evaluate its effect on the improvement of long-term survival.
Results: There were 11 t(1;19)-positive cases documented by karyotyping and 3 E2A-PBX1 transcript-positive cases amplified by PCR from the initial bone marrow samples. There were 11 cases of FAB L1 and 3 cases of L2. Immunophenotypic
classification revealed 10 cases of group Ⅴ, 2 cases of group Ⅳ, and 1 case of group Ⅲ. Among 11 cases with documented karyotype, 9 cases (81.8%) had der(19)t(1;19) and the other 2 had balanced t(1;19). Fifty-six samples collected at 2 to 7
different
time points of 14 patients revealed 4 cases of MRD immediately after completion of induction chemotherapy despite hematologic complete remission. Three of these cases relapsed eventually, and 1 was lost to follow-up. Among 12 cases who received
adequate
delayed intensificiation chemotherapy, 10 are alive in complete remission.
Conclusion: MRD detection by RT-PCR amplification of E2A-PBX1 transcript of induction chemotherapy was most informative for the prediction of long-term survival and relapse. The presence of MRD after completion of induction chemotherapy
conferred
poor prognosis. The addition of delayed intensification chemotherapy to standard chemotherapy regimen could abolish the adverse effect of t(1;19) in childhood ALL patients.
Keywords: E2A-PBX1, t(1,19), Minimal residual disease, Childhood ALL, Chemotherapy,
Previous Article
