Complex and monosomal karyotype are distinct cytogenetic entities with an adverse prognostic impact in paediatric acute myeloid leukaemia. A NOPHO NOPHO ‐ DBH DBH ‐ AML AML study

摘要

Summary Data on occurrence, genetic characteristics and prognostic impact of complex and monosomal karyotype ( CK / MK ) in children with acute myeloid leukaemia ( AML ) are scarce. We studied CK and MK in a large unselected cohort of childhood AML patients diagnosed and treated according to Nordic Society for Paediatric Haematology and Oncology ( NOPHO )‐ AML protocols 1993–2015. In total, 800 patients with de novo AML were included. CK was found in 122 (15%) and MK in 41 (5%) patients. CK and MK patients were young (median age 2·1 and 3·3?years, respectively) and frequently had FAB M7 morphology (24% and 22%, respectively). Refractory disease was more common in MK patients (15% vs. 4%) and stem cell transplantation in first complete remission was more frequent (32% vs. 19%) compared with non‐ CK /non‐ MK patients. CK showed no association with refractory disease but was an independent predictor of an inferior event‐free survival ( EFS ; hazard ratio [ HR ] 1·43, P ?=?0·03) and overall survival ( OS ; HR 1·48, P ?=?0·01). MK was associated with a poor EFS ( HR 1·57, P ?=?0·03) but did not show an inferior OS compared to non‐ MK patients ( HR 1·14, P ?=?0·62). In a large paediatric cohort, we characterized AML with non‐recurrent abnormal karyotype and unravelled the adverse impact of CK and MK on prognosis.