Unusually Long Survival of a 67-Year-Old Patient with Near-Tetraploid Acute Myeloid Leukemia M0 without Erythroblastic and Megakaryocytic Dysplasia
Abstract
Patients with near-tetraploid acute myeloid leukemia (NT-AML) typically have poor survival. We present the case of a 67-year-old Caucasian male with NT-AML M0 who had an unusually long first complete remission of 51 months and an overall survival of 80 months.
The only characteristic distinguishing him from other previously described patients with NT-AML was the absence of erythroblastic and/or megakaryocytic dysplasia (EMD) at diagnosis. Molecular-genetic testing for AML fusion transcripts associated with a favorable prognosis (PML/RAR alpha, AML1/ETO, and CBF beta/MYH11) were negative, as were other prognostic markers like MLL-PTD, FLT3-ITD, or mutations of FLT3-D835, NPM1, or CEBPA.
Expression studies of ERG, MN1, and EVI1 revealed overexpression of ERG only. The absence of EMD may be a useful prognostic/diagnostic feature of this new rare subtype of NT-AML.