TY - JOUR A1 - Rasche, Mareike A1 - Steidel, Emma A1 - Zimmermann, Martin A1 - Bourquin, Jean-Pierre A1 - Boztug, Heidrun A1 - Janotova, Iveta A1 - Kolb, E. Anders A1 - Lehrnbecher, Thomas A1 - Neuhoff, Nils von A1 - Niktoreh, Naghmeh A1 - Mühlegger, Nora A1 - Sramkova, Lucie A1 - Stary, Jan A1 - Walter, Christiane A1 - Creutzig, Ursula A1 - Dworzak, Michael A1 - Reinhardt, Dirk T1 - Second relapse of pediatric patients with acute myeloid leukemia: a report on current treatment strategies and outcome of the AML-BFM study group T2 - Cancers N2 - Simple Summary: Children with acute myeloid leukemia (AML) experience high relapse rates of about 30%; still, survival rates following the first relapse are encouraging. Hence, it is critically important to examine the consequences of a second relapse; however, little is known about this subgroup of patients. This retrospective population-based analysis intends to describe response, survival and prognostic factors relevant for the survival of children with second relapse of AML. Treatment approaches include many different therapeutic regimens, including palliation and intensive treatment with curative intent (63% of the patients). Survival is poor; however, patients who respond to reinduction attempts can be rescued with subsequent hematopoietic stem cell transplantation. We deciphered risk factors, such as short time interval from first to second relapse below one year as being associated with a poor outcome. This analysis will help to improve future international treatment planning and patient care of children with advanced AML. Abstract: Successful management of relapse is critical to improve outcomes of children with acute myeloid leukemia (AML). We evaluated response, survival and prognostic factors after a second relapse of AML. Among 1222 pediatric patients of the population-based AML-Berlin–Frankfurt–Munster (BFM) study group (2004 until 2017), 73 patients met the quality parameters for inclusion in this study. Central review of source documentation warranted the accuracy of reported data. Treatment approaches included palliation in 17 patients (23%), intensive therapy with curative intent (n = 46, 63%) and other regimens (n = 10). Twenty-five patients (35%) received hematopoietic stem cell transplantation (HSCT), 21 of whom (88%) had a prior HSCT. Survival was poor, with a five-year probability of overall survival (pOS) of 15 ± 4% and 31 ± 9% following HSCT (n = 25). Early second relapse (within one year after first relapse) was associated with dismal outcome (pOS 2 ± 2%, n = 44 vs. 33 ± 9%, n = 29; p < 0.0001). A third complete remission (CR) is required for survival: 31% (n = 14) of patients with intensive treatment achieved a third CR with a pOS of 36 ± 13%, while 28 patients (62%) were non-responders (pOS 7 ± 5%). In conclusion, survival is poor but possible, particularly after a late second relapse and an intensive chemotherapy followed by HSCT. This analysis provides a baseline for future treatment planning. KW - acute myeloid leukemia KW - relapse KW - childhood acute myeloid leukemia KW - pediatric patients KW - salvage therapy Y1 - 2021 UR - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/62154 UR - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-621546 SN - 2072-6694 N1 - This work was supported by the German Cancer Aid 50-2728, 110244. M.R. is supported by the Josepha und Charlotte von Siebold program. VL - 13 IS - 4, art. 789 SP - 1 EP - 13 PB - MDPI CY - Basel ER -