Expression of mutant NFE2 causes AML with concomitant granulocytic sarcoma as well as isolated granulocytic sarcoma in mice. (A) Diagnoses according to the Bethesda Classification of Nonlymphoid Hematological Malignancies10 in n = 41 mice expressing various NFE2 mutations9 (B-E) Protein nomenclature of indicated NFE2 mutants, s. supplemental Table 2. (B) Hematological parameters of selected mice that developed leukemia. WBC: white blood cells; HCT, hematocrit; PLT, platelets. Blood counts indicating leukocytosis, anemia, and thrombocytopenia, the clinical hallmarks of leukemia, are highlighted in the black boxes. (C) FACS analysis of PB leukocytes of mouse #539 and #40. Cells were stained with CD11b and Ly6G/C or CD3ε and CD45R to determine myeloid and lymphoid cells, respectively. (D) From left to right: PB smear of mouse #539 (1000×) and chloracetate esterase (CAE) stained femur section of mouse #40 (400× and 1000×). (E) Histological analysis, hematoxylin and eosin–stained sections of the following organs of leukemic mice: #539 (from left to right): femur (1000×), liver (1000×), kidney (10× and 1000×). #535 (from left to right): femur (1000×), kidney (10× and 1000×). #537 (from left to right): femur (400× and 1000×), lung (400×). #40 (from left to right): sarcoma (400×), lung (400× and 1000×). #32 (from left to right): top: femur (400× and 1000×), liver (400× and 1000×); bottom: lung (25× and 1000×). Bars: 200 μm (10×), 500 μm (25×), 50 μm (400×), 20 μm (1000×).