BACKGROUND: The prognosis of patients with relapses of ETV6/RUNX1-positive acute lymphoblastic leukemia remains to be evaluated, particularly with regards to the frequency of late relapses. We performed a long-term, follow-up retrospective study to address the outcome of patients with ETV6/RUNX1-positive leukemia relapses. DESIGN AND METHODS: Among the 713 children tested for ETV6/RUNX1 enrolled into the FRALLE 93 protocol, 43 ETV6/RUNX1-positive patients relapsed (19.4%). Most were initially stratified in the low or intermediate risk groups. The median follow-up after relapse was 54.2 months. All but three received second-line salvage therapy and 16 underwent allogeneic transplantation. RESULTS: ETV6/RUNX1 had a strong effect on overall survival after relapse (3-year survival= 64.7% for positive cases versus 46.5% for negative cases) (P=0.007). The 5-year cumulative incidence of relapse was 19.4% and testes were more frequently involved in ETV6/RUNX1-positive relapses (P=0.04). In 81.4% of cases the relapses were late, early combined or isolated extramedullary relapses. The 5-year survival rate of patients with ETV6-RUNX1-positive acute lymphoblastic leukemia relapses reached 80.8% when the relapse occurred after 36 months (versus 31.2% when the relapse occurred earlier). In univariate analysis, female gender was associated with a poor survival, whereas site of relapse, age at diagnosis, leukocytosis and consolidation strategy had no effect. In multivariate analysis, only the duration of first remission remained associated with outcome. CONCLUSIONS: We found an excellent outcome for patients with ETV6/RUNX1-positive leukemia relapses that occurred more than 36 months after diagnosis. The duration of first complete remission may, therefore, be a guide to define the treatment strategy for patients with relapsed ETV6/RUNX1- positive leukemia.
BACKGROUND: The prognosis of patients with relapses of ETV6/RUNX1-positive acute lymphoblastic leukemia remains to be evaluated, particularly with regards to the frequency of late relapses. We performed a long-term, follow-up retrospective study to address the outcome of patients with ETV6/RUNX1-positive leukemia relapses. DESIGN AND METHODS: Among the 713 children tested for ETV6/RUNX1 enrolled into the FRALLE 93 protocol, 43 ETV6/RUNX1-positive patients relapsed (19.4%). Most were initially stratified in the low or intermediate risk groups. The median follow-up after relapse was 54.2 months. All but three received second-line salvage therapy and 16 underwent allogeneic transplantation. RESULTS:ETV6/RUNX1 had a strong effect on overall survival after relapse (3-year survival= 64.7% for positive cases versus 46.5% for negative cases) (P=0.007). The 5-year cumulative incidence of relapse was 19.4% and testes were more frequently involved in ETV6/RUNX1-positive relapses (P=0.04). In 81.4% of cases the relapses were late, early combined or isolated extramedullary relapses. The 5-year survival rate of patients with ETV6-RUNX1-positive acute lymphoblastic leukemia relapses reached 80.8% when the relapse occurred after 36 months (versus 31.2% when the relapse occurred earlier). In univariate analysis, female gender was associated with a poor survival, whereas site of relapse, age at diagnosis, leukocytosis and consolidation strategy had no effect. In multivariate analysis, only the duration of first remission remained associated with outcome. CONCLUSIONS: We found an excellent outcome for patients with ETV6/RUNX1-positive leukemia relapses that occurred more than 36 months after diagnosis. The duration of first complete remission may, therefore, be a guide to define the treatment strategy for patients with relapsed ETV6/RUNX1- positive leukemia.
Authors: F J T Staal; D de Ridder; T Szczepanski; T Schonewille; E C E van der Linden; E R van Wering; V H J van der Velden; J J M van Dongen Journal: Leukemia Date: 2010-01-14 Impact factor: 11.528
Authors: A Möricke; M Zimmermann; A Reiter; G Henze; A Schrauder; H Gadner; W D Ludwig; J Ritter; J Harbott; G Mann; T Klingebiel; F Zintl; C Niemeyer; B Kremens; F Niggli; D Niethammer; K Welte; M Stanulla; E Odenwald; H Riehm; M Schrappe Journal: Leukemia Date: 2009-12-10 Impact factor: 11.528
Authors: Elizabeth A Raetz; Michael J Borowitz; Meenakshi Devidas; Stephen B Linda; Stephen P Hunger; Naomi J Winick; Bruce M Camitta; Paul S Gaynon; William L Carroll Journal: J Clin Oncol Date: 2008-08-20 Impact factor: 44.544
Authors: Peter Bader; Hermann Kreyenberg; Günter H R Henze; Cornelia Eckert; Miriam Reising; Andre Willasch; Andrea Barth; Arndt Borkhardt; Christina Peters; Rupert Handgretinger; Karl-Walter Sykora; Wolfgang Holter; Hartmut Kabisch; Thomas Klingebiel; Arend von Stackelberg Journal: J Clin Oncol Date: 2008-12-08 Impact factor: 44.544
Authors: L B Silverman; K E Stevenson; J E O'Brien; B L Asselin; R D Barr; L Clavell; P D Cole; K M Kelly; C Laverdiere; B Michon; M A Schorin; C L Schwartz; E W O'Holleran; D S Neuberg; H J Cohen; S E Sallan Journal: Leukemia Date: 2009-12-17 Impact factor: 11.528
Authors: C H Pui; D Pei; J T Sandlund; R C Ribeiro; J E Rubnitz; S C Raimondi; M Onciu; D Campana; L E Kun; S Jeha; C Cheng; S C Howard; M L Metzger; D Bhojwani; J R Downing; W E Evans; M V Relling Journal: Leukemia Date: 2009-12-10 Impact factor: 11.528
Authors: A Alsadeq; S Strube; S Krause; M Carlet; I Jeremias; C Vokuhl; S Loges; J A Aguirre-Ghiso; A Trauzold; G Cario; M Stanulla; M Schrappe; D M Schewe Journal: Leukemia Date: 2015-06-24 Impact factor: 11.528
Authors: Ameera Alsadeq; Henning Fedders; Christian Vokuhl; Nele M Belau; Martin Zimmermann; Tim Wirbelauer; Steffi Spielberg; Michaela Vossen-Gajcy; Gunnar Cario; Martin Schrappe; Denis M Schewe Journal: Haematologica Date: 2016-09-29 Impact factor: 9.941