The histiocytic disorders comprise a broad grouping of hematologic and immunologic diseases united by aberrant function, differentiation, and/or proliferation of cells of the mononuclear phagocyte system. The term histiocyte historically referred to tissue phagocytes but now more precisely defines cells…
The histiocytic disorders comprise a broad grouping of hematologic and immunologic diseases united by aberrant function, differentiation, and/or proliferation of cells of the mononuclear phagocyte system. The term histiocyte historically referred to tissue phagocytes but now more precisely defines cells…
Introduction Langerhans cell histiocytosis and related neoplastic histiocytoses (Erdheim–Chester disease, juvenile xanthogranuloma, and Rosai–Dorfman disease) typically arise from clonal proliferation of myeloid precursor cells driven by pathologically activated MAPK signaling, where hemophagocytic lymphohistiocytosis is a heterogeneous syndrome characterized by excessive…