Third consecutive national study ALL-BFM 95 improved the outcome of acute lymphoblastic leukemia in children in the Czech Republic


Authors: J. Starý 1;  P. Gajdoš 1;  H. Hrstková 2;  L. Kopečná 2;  J. Štěrba 3;  D. Dembická 3;  D. Mendelová 3;  D. Pospíšilová 4;  M. Hajdůch 4;  B. Blažek 5;  H. Ptoszková 5;  J. Hak 6;  K. Toušovská 6;  D. Procházková 7;  Z. Černá 8;  Y. Jabali 9;  P. Timr 9;  P. Smíšek 1;  V. Vávra 1;  K. Zdráhalová 1;  P. Sedláček 1;  L. Šrámková 1;  V. Komrska 1;  E. Mejstříková 1;  A. Vrzalová 1;  K. Kramarzová 1;  K. Michalová 10;  Z. Zemanová 10;  M. Jarošová 11;  J. Housková 12;  J. Zuna 1;  O. Hrušák 1;  J. Trka 1;  V. Mihál 4
Authors‘ workplace: Klinika dětské hematologie a onkologie UK 2. LF a FN Motol, Praha, 2I. dětská interní klinika LF MU a FN Brno, 3Klinika dětské onkologie FN Brno, 4Dětská klinika LF UP a FN Olomouc, 5Klinika dětského lékařství FN Ostrava, 6Dětská klinika FN Hradec Králové 1
Published in: Transfuze Hematol. dnes,14, 2008, No. 3, p. 102-109.
Category: Comprehensive Reports, Original Papers, Case Reports

Overview

Treatment of childhood acute lymphoblastic leukemia (ALL) according to the protocol ALL-BFM 95 was performed at 8 departments of pediatrics between 1996–2002 in the Czech Republic. Patients were stratified into 3 risk groups (standard risk-SR, medium risk-MR and high risk-HR) according to the early treatment response, age, initial WBC and the finding of translocations t(9;22) or t(4;11). Total duration of treatment was 2 years except for boys of SR for whom it was three years. 386 children were enrolled into the study and the treatment results were evaluable in 380 patients with a median follow-up of 8.2 years. Complete remission (CR) was achieved in 95.5% pts, 3.9% children died in CR. Stratification into the risk groups was as follows: SR 32.4%, MR 53.7% and HR 13.9%. 69 children (19%) suffered from relapse, isolated CNS relapse has been diagnosed only in 1.1%. Two children (0.6%) developed secondary cancer. Event-free survival (EFS) is 73.1% and overall survival (OS) 80.8%. The results improved in comparison with previous study ALL-BFM 90 (EFS 71.3%; OS 76.4%) despite treatment reduction for 2/3 of patients. EFS/OS were in SR 84.5/93.2%, MR 74/82.1% and HR 43.1/47.1%. The prognosis of T-ALL (EFS 64.8%) was significantly worse in comparison with BCP-ALL (EFS 75.8%). Only 7 children underwent stem cell transplantation in the first CR. Survival of children with ALL in the Czech Republic exceeded 80%. Significant improvement was particularly achieved in HR group (EFS in previous study only 28.2%).

Key words:
acute lymphoblastic leukemia, children, chemotherapy, protocol ALL-BFM 95, relapse


Sources

1. Starý J, Gajdoš P, Blažek B, et al. Zlepšení výsledků léčby dětí s akutní lymfoblastickou leukemií podle protokolu ALL-BFM 90 v České republice. Čas Lék čes 2003; 142: 404–409.

2. Starý J a Pracovní skupina pro dětskou hematologii České republiky. Léčba dětí s akutní lymfoblastickou leukemií v České republice podle protokolu ALL-BFM 83. Čs Pediatr 1993; 48: 391–397.

3. Starý J, Gajdoš P, Trka J, et al. Akutní lymfoblastická leukemie u kojenců. Transfuz Hemat dnes 2002; 8: 47–54.

4. Hrušák O, Trka J, Zuna J, et al. Aberrant expression of KOR-SA3544 antigen in childhood acute lymphoblastic leukemia predicts TEL-AML1 negativity. Leukemia 1998; 12: 1064–1070.

5. van-der-Does van-den-Berg A, Bartram CR, Basso G, et al. Minimal requirements for the diagnosis, classification, and evaluation of the treatment of childhood acute lymphoblastic leukemia (ALL) in the „BFM Family“ Cooperative Group. Med Pediatr Oncol 1992; 20: 497–505.

6. European group for the immunological characterisation of leukaemias (EGIL), Bene MC, Castoldi G, Knapp W, et al. Proposals for the immunological classification of acute leukaemias. Leukemia 1995; 9: 1783–1786.

7. Zemanová Z, Michalová K, Šindelářová L, et al. Prognostic value of structural chromosomal rearrangements and small cell clones with high hyperdiploidy in children with acute lymphoblastic leukemia. Leuk Res 2005; 29: 273–281.

8. Trka J, Zuna J, Haškovec C, et al. Detekce hybridních genů BCR/ABL, MLL/AF4 a TEL/AML1 a sledování minimální residuální nemoci u dětských pacientů s akutní lymfoblastickou leukemií. Čas Lék čes 1999; 138: 12–17.

9. Kaplan EL, Meier P. Nonparametric estimation from incomplete observations. J Am Stat Assoc 1958; 53: 457–481.

10. Mantel N. Evaluation of survival data and two new rank order statistics arising in its consideration. Cancer Chemother Rep 1966; 50: 163–170.

11. Conter V, Valsecchi MG, Silvestri D, et al. Pulses of vincristine and dexamethasone in addition to intensive chemotherapy for children with intermediate-risk acute lymphoblastic leukaemia: a multicentre randomised trial. Lancet 2007; 369: 123–31.

12. Zuna J, Hrušák O, Kalinová M, et al. Significantly lower relapse rate for TEL/AML1-positive ALL. Leukemia 1999; 13: 1633–38.

13. Seeger K, Buchwald D, Taube T, et al. TEL-AML1 positivity in relapsed B cell precursor acute lymphoblastic leukemia in childhood. Leukemia 1999; 13: 1469–1470.

14. De Labarthe A, Rousselot P, Huguet-Rigal F, et al. Imatinib combined with induction or consolidation chemotherapy in patiens with de novo Philadelphia chromosome-positive acute lymphoblastic leukemia: results of the GRAAPH-2003 study. Blood 2007; 109: 1408–1413.

15. Balduzzi A, Valsecchi MG, Uderzo C, et al. Chemotherapy versus allogeneic transplantation for very-high risk childhood acute lymphoblastic leukaemia in first complete remission: comparison by genetic randomisation in an international prospective study. Lancet 2005; 366: 635–642.

16. Whitlock JA, Sather HN, Gaynon P, et al Clinical characteristics and outcome of children with Down syndrome and acute lymphoblastic leukemia: a Children’s Cancer Group study. Blood 2005; 106: 4043–4049.

17. Möricke A, Reiter A, Zimmermann M, et al. Risk-adjusted therapy of acute lymphoblastic leukemia can decrease treatment burden and improve survival: treatment results of 2169 unselected pediatric and adolescent patiens enrolled in the trial ALL-BFM 95. Blood 2008; 111: 4477–89.

Labels
Haematology Internal medicine Clinical oncology
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