A 47-year-old female presented with neutropenic fever. Hb 131 g/L, WBC 15.6 x10^9/L, neutrophil 0.16 x10^9/L, platelet 36 x10^9/L. Bone marrow aspirate showed monocytoid blasts with occasional Auer rods. By flow cytometry, blasts were positive for CD34, CD117, HLADR, CD33, CD7, CD13, CD11b, CD56, CD15wk, cMPO and negative for cTdT, cCD3, and cCD79a. Myeloid NGS showed CEBPA p.Glu89Ter (VAF 46%), CEBPA p.Lys304_Arg306dup (VAF 38%) and WT1 p.Ser386Ter (VAF 8%). No fusions were detected. Targeted FISH showed an isolated 16q loss. She was treated with daunorubicin, cytarabine and gemtuzumab.

AML with a biallelic CEBPA mutation with biZIP has a favourable prognosis. The leukemic cells are often positive for CD7, but they rarely express CD56 or CD14.