Figure 1
(A) Simplified depiction of Ras protein with sites of mutation noted in Table 1. Combined structures of KRAS (blue) and NRAS (magenta). The mutation on the right (closest to bound guanosine diphosphate [GDP]) is G13, and G12 is on the left (farthest from bound GDP). (B) Peripheral blood monocytes from 8-year-old RALD patient 381.1 with leukocytosis, monocytosis, and lymphocytosis (×1000). (C) Bone marrow aspirate from same patient showing granulocytic hyperplasia with mild-to-moderate left shift in myeloid maturation with less than 5% blasts (×500). (D) Bone marrow from patient 104.1 showing pelgeroid granulocytes and left shift. (E) Healthy control peripheral blood (PB) with normal circulating monocytes that are CD16–. (F) Healthy control PB showing that only monocytes (turquoise) are positive for CD14 and granulocytes (green population) are negative for CD14. (G) Healthy control PB lymphocyte gate demonstrating that CD20+ B cells are largely CD10–. (H) PB CD14+ monocyte gate showing increased expression of CD16 on monocytes in RALD in comparison with monocytes from healthy control (E). (I) Prominent increased expression of CD14 on granulocytes (green) in RALD which is not seen in healthy controls (F). (J) Peripheral blood lymphocyte gate with B-cell lymphocytosis (polyclonal) with marked increase in CD10+ circulating late precursor B cells in comparison with healthy control (G).

(A) Simplified depiction of Ras protein with sites of mutation noted in Table 1. Combined structures of KRAS (blue) and NRAS (magenta). The mutation on the right (closest to bound guanosine diphosphate [GDP]) is G13, and G12 is on the left (farthest from bound GDP). (B) Peripheral blood monocytes from 8-year-old RALD patient 381.1 with leukocytosis, monocytosis, and lymphocytosis (×1000). (C) Bone marrow aspirate from same patient showing granulocytic hyperplasia with mild-to-moderate left shift in myeloid maturation with less than 5% blasts (×500). (D) Bone marrow from patient 104.1 showing pelgeroid granulocytes and left shift. (E) Healthy control peripheral blood (PB) with normal circulating monocytes that are CD16. (F) Healthy control PB showing that only monocytes (turquoise) are positive for CD14 and granulocytes (green population) are negative for CD14. (G) Healthy control PB lymphocyte gate demonstrating that CD20+ B cells are largely CD10. (H) PB CD14+ monocyte gate showing increased expression of CD16 on monocytes in RALD in comparison with monocytes from healthy control (E). (I) Prominent increased expression of CD14 on granulocytes (green) in RALD which is not seen in healthy controls (F). (J) Peripheral blood lymphocyte gate with B-cell lymphocytosis (polyclonal) with marked increase in CD10+ circulating late precursor B cells in comparison with healthy control (G).

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