Zurück zur Übersicht
| Haematologica. 2019 Oct 10. pii: haematol.2019.231720. doi: 10.3324/haematol.2019.231720 |
| Relapses and treatment-related events contributed equally to poor prognosis in children with ABL-class fusion positive B-cell acute lymphoblastic leukemia treated according to AIEOP-BFM protocols. |
| Cario G1, Leoni V2, Conter V3, Attarbaschi A4, Zaliova M5, Sramkova L6, Cazzaniga G7, Fazio G8, Sutton R9, Elitzur S10, Izraeli S11, Lauten M12, Locatelli F13, Basso G14, Buldini B15, Bergmann AK16, Lentes J17, Steinemann D18, Göhring G19, Schlegelberger B20, Haas OA21, Schewe D22, Buchmann S23, Moericke A24, White D25, Revesz T26, Stanulla M27, Mann G28, Bodmer N29, Arad-Cohen N30, Zuna J31, Valsecchi MG32, Zimmermann M33, Schrappe M34, Biondi A35 |
 Author information
|
|
Abstract ABL-class fusions other than BCR-ABL1 characterize about 2-3% of precursor B-cell acute lymphoblastic leukemia. Case series indicated that patients suffering from these subtypes have a dismal outcome and may benefit from the introduction of tyrosine kinase inhibitors. We analyzed clinical characteristics and outcome of 46 ABL-class fusion positive cases other than BCR-ABL1 treated according to AIEOP-BFM ALL 2000 and 2009 protocols; 13 of them received a tyrosine kinase inhibitor during different phases of treatment. ABL-class fusion positive cases had a poor early treatment response: minimal residual disease levels of >5x10-4 were observed in 71.4% of patients after induction treatment and in 51.2% after consolidation phase. For the entire cohort of 46 cases the 5-year probability of event-free survival was 49.1+8.9% and that of overall survival 69.6+7.8%; the cumulative incidence of relapse was 25.6+8.2% and treatment-related mortality 20.8+6.8%. One out of 13 cases with tyrosine kinase inhibitor added to chemotherapy relapsed while eight of 33 cases without tyrosine kinase inhibitor treatment suffered from relapse, including six in 17 patients who had not received hematopoietic stem cell transplantation. Stem cell transplantation seems to be effective in preventing relapses (only three relapses in 25 patients), but was associated with a very high treatment-related mortality (6 patients). These data indicate a major need for an early identification of ABL-class fusion positive acute lymphoblastic leukemia cases and to establish a properly designed, controlled study aimed at investigating the use of tyrosine kinase inhibitors, the appropriate chemotherapy backbone and the role of hematopoietic stem cell transplantation. (ClinicalTrials.gov identifier: NTC00430118, NCT00613457, NCT01117441). |
|
Copyright © 2019, Ferrata Storti Foundation. |
|
KEYWORDS: ABL-class fusion, Pediatric Acute Lymphoblastic Leukemia, Tyrosin Kinase Inhibitors |
|
Publikations ID: 31601692 Quelle: öffnen |
Author information
Schnellinfo
-- Cancer Care
-- Kliniken und Partner
-- CCC Cancer School
-- Young CCC
-- CCC Tumorboards
-- CCC Forschungscluster
-- CCC Units
-- CCC Platforms
-- SOPs / Leitlinien
-- Kontakt
Featured
