BACKGROUND: The t(9;22) and t(4;11) chromosomal translocations, which generate the BCR-ABL and MLL-AF4 fusion genes, define high-risk subtypes of acute lymphoblastic leukemia in adults. However, the prognostic impact of other rarer fusion genes is less well established in adult acute lymphoblastic leukemia than in the childhood form. DESIGN AND METHODS: In the context of the German Multicenter Therapy Study Group for Adult Acute Lymphoblastic Leukemia (GMALL) we used reverse transcriptase polymerase chain reaction to investigate 441 cases of BCR-ABL- and MLL-AF4-negative B-precursor acute lymphoblastic leukemia for the TCF3-PBX1 (E2A-PBX1) and ETV6-RUNX1 (TEL-AML1) fusion transcripts generated by the t(1;19)(q23;p13.3) and t(12;21)(p13;q22) translocations. Both are well-known molecular alterations in pediatric acute lymphoblastic leukemia in which they have favorable prognostic implications. RESULTS: We identified 23 adult patients with TCF3-PBX1 and ten with ETV6-RUNX1. In contrast to previous reports we found no significant difference in overall survival between TCF3-PBX1-positive and -negative patients. At 2 years after diagnosis all the ETV6-RUNX1-positive patients were alive and in continuous complete remission, but their long-term outcome was negatively affected by late relapses. TCF3-PBX1-positive patients exhibited a characteristic CD34(-)/CD33(-) and mostly cyIg(+) immunophenotype. ETV6-RUNX1 only occurred in patients under 35 years old and was associated with a significantly lower white blood count. CONCLUSIONS: In contrast to previous suggestions, adult patients with TCF3-PBX1-positive acute lymphoblastic leukemia do not appear to have a worse outcome than their negative counterparts. ETV6-RUNX1-positive patients had a very favorable performance status during the first few years but their long-term survival was negatively affected by late relapses. Both groups of patients are characterized by distinct clinicobiological features which facilitate their diagnostic identification.
BACKGROUND: The t(9;22) and t(4;11) chromosomal translocations, which generate the BCR-ABL and MLL-AF4 fusion genes, define high-risk subtypes of acute lymphoblastic leukemia in adults. However, the prognostic impact of other rarer fusion genes is less well established in adult acute lymphoblastic leukemia than in the childhood form. DESIGN AND METHODS: In the context of the German Multicenter Therapy Study Group for Adult Acute Lymphoblastic Leukemia (GMALL) we used reverse transcriptase polymerase chain reaction to investigate 441 cases of BCR-ABL- and MLL-AF4-negative B-precursor acute lymphoblastic leukemia for the TCF3-PBX1 (E2A-PBX1) and ETV6-RUNX1 (TEL-AML1) fusion transcripts generated by the t(1;19)(q23;p13.3) and t(12;21)(p13;q22) translocations. Both are well-known molecular alterations in pediatric acute lymphoblastic leukemia in which they have favorable prognostic implications. RESULTS: We identified 23 adult patients with TCF3-PBX1 and ten with ETV6-RUNX1. In contrast to previous reports we found no significant difference in overall survival between TCF3-PBX1-positive and -negative patients. At 2 years after diagnosis all the ETV6-RUNX1-positive patients were alive and in continuous complete remission, but their long-term outcome was negatively affected by late relapses. TCF3-PBX1-positive patients exhibited a characteristic CD34(-)/CD33(-) and mostly cyIg(+) immunophenotype. ETV6-RUNX1 only occurred in patients under 35 years old and was associated with a significantly lower white blood count. CONCLUSIONS: In contrast to previous suggestions, adult patients with TCF3-PBX1-positive acute lymphoblastic leukemia do not appear to have a worse outcome than their negative counterparts. ETV6-RUNX1-positive patients had a very favorable performance status during the first few years but their long-term survival was negatively affected by late relapses. Both groups of patients are characterized by distinct clinicobiological features which facilitate their diagnostic identification.
Authors: M L Martín Ramos; J J Lahuerta Palacios; J Martínez López; M J Gómez Rodríguez; A Moreno Izquierdo; E Barreiro Miranda Journal: Haematologica Date: 2001-04 Impact factor: 9.941
Authors: A Andersson; M Höglund; B Johansson; C Lassen; R Billström; S Garwicz; P G Nilsson; F Mitelman; T Fioretos Journal: Leukemia Date: 2001-08 Impact factor: 11.528
Authors: Beate Gleissner; Nicola Gökbuget; Claus R Bartram; Bart Janssen; Harald Rieder; Johannes W G Janssen; Christa Fonatsch; Axel Heyll; Dimitris Voliotis; Joachim Beck; Thomas Lipp; Gerd Munzert; Jürgen Maurer; Dieter Hoelzer; Eckhard Thiel Journal: Blood Date: 2002-03-01 Impact factor: 22.113
Authors: J J van Dongen; E A Macintyre; J A Gabert; E Delabesse; V Rossi; G Saglio; E Gottardi; A Rambaldi; G Dotti; F Griesinger; A Parreira; P Gameiro; M G Diáz; M Malec; A W Langerak; J F San Miguel; A Biondi Journal: Leukemia Date: 1999-12 Impact factor: 11.528
Authors: M S Jabber Al-Obaidi; M Martineau; C F Bennett; I M Franklin; A H Goldstone; L Harewood; G R Jalali; H G Prentice; S M Richards; K Roberts; C J Harrison Journal: Leukemia Date: 2002-04 Impact factor: 11.528
Authors: K Seeger; K A Kreuzer; U Lass; T Taube; D Buchwald; C Eckert; G Körner; C A Schmidt; G Henze Journal: Cancer Res Date: 2001-03-15 Impact factor: 12.701
Authors: S Jeha; D Pei; S C Raimondi; M Onciu; D Campana; C Cheng; J T Sandlund; R C Ribeiro; J E Rubnitz; S C Howard; J R Downing; W E Evans; M V Relling; C-H Pui Journal: Leukemia Date: 2009-03-12 Impact factor: 11.528