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Multiple Choice

Which diagnosis fits neutropenia with hypogranulated and hyposegmented neutrophils and abnormal myeloid maturation on marrow with high ferritin?

Hyperinflammation with macrophage activation and hemophagocytosis explains this pattern. In hemophagocytic lymphohistiocytosis, cytopenias come from immune-driven destruction and phagocytosis of blood cells, and the marrow often shows abnormal myeloid maturation under the influence of inflammatory cytokines. Marked ferritin elevation is a hallmark of macrophage activation and disease severity. While dysmyelopoiesis or dysmegakaryocytopoiesis describe dysplasia in specific cell lines and myelodysplastic syndromes involve clonal marrow abnormalities, they don’t typically feature the same degree of ferritin elevation or the macrophage-driven hemophagocytic process. The combination of neutropenia with hypogranulated and hyposegmented neutrophils, abnormal myeloid maturation on marrow, and very high ferritin best fits hemophagocytic lymphohistiocytosis.

Hyperinflammation with macrophage activation and hemophagocytosis explains this pattern. In hemophagocytic lymphohistiocytosis, cytopenias come from immune-driven destruction and phagocytosis of blood cells, and the marrow often shows abnormal myeloid maturation under the influence of inflammatory cytokines. Marked ferritin elevation is a hallmark of macrophage activation and disease severity. While dysmyelopoiesis or dysmegakaryocytopoiesis describe dysplasia in specific cell lines and myelodysplastic syndromes involve clonal marrow abnormalities, they don’t typically feature the same degree of ferritin elevation or the macrophage-driven hemophagocytic process. The combination of neutropenia with hypogranulated and hyposegmented neutrophils, abnormal myeloid maturation on marrow, and very high ferritin best fits hemophagocytic lymphohistiocytosis.