Extramedullary acute myeloid leukemia (eAML): Retrospective single center cohort study on clinico-pathological, molecular analysis and survival outcomes

Myeloid sarcoma CD117 Single Center
DOI: 10.1016/j.amsu.2021.102894 Publication Date: 2021-10-22T14:00:16Z
ABSTRACT
extramedullary acute myeloid leukemia (eAML) is characterized by tumor formation infiltrated blasts, with or without maturation and effaced architecture. The clinical, genetic molecular aspects overall outcomes are well defined worldwide, but not in our region.This a retrospective single center cohort study on 32 patients, who were identified over 10 years to the pathologic genetic-molecular aspects, survival outcomes.eAML rare (1%), occurs at younger age male predominance. Central nervous system (CNS) facial bone invasion most commonly (34.4%). 45.5% positive for conventional markers (MPO), CD33, CD117, 36% CD34 CD68. 54% normal karyotype had deleterious mutations further testing. NGS revealed pathogenic 76%(N-9/17) none tested P53, IDH1 IDH2. At median follow up time of 43mo (range, 8.6-80mo); 37.5%(N-12) complete remission, 62.5%(N-20) relapsed. 28% relapses after allotransplant. 31%(N-10) alive continued remission(CR), 69%(N-22) patients have died.Median (OS) 18.4 relapse free (RFS) 18.7 months. OS RFS significantly better attained CR induction (IC 11.9 mo vs zero; P = 0.0001; IC 12mo 0.0001) compared relapsed disease; received allo-transplant consolidation 42 8.5mo (P 0.002) 42months 0.006). Thus allotransplant may be considered all eligible first CR.achievement remission therapy associated improved eAML. Allotransplant effective modality achieving long-term remissions.
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