Autoimmune-Associated Hemophagocytosis and Myelofibrosis in a Newly Diagnosed Lupus Patient: Case Report and Literature Review

Figure 4

Possible pathogenesis of coexisting hemophagocytosis and myelofibrosis in SLE. The state of immune activation in SLE leads to excessive production of cytokines such as IFN-γ, TNF-α, and IL-6. TNF-α is known to induce activation of macrophages. AAHS occurs as activated macrophages engulf mature and hematopoietic stem cells and further contribute to cytokine production. TGF-β, which is a cytokine produced by activated macrophages, stimulates fibroblast proliferation leading to AIMF.