Autoimmune-associated hemophagocytic syndrome originating from autoimmune hepatitis with a successful response to therapy. Saito M1, Yano Y, Minami A, Momose K, Hirano H, Yamasaki T, Itoh T, Yoshida M, Azuma T.
Accordingly, autoimmune-associated hemophagocytic syndrome (AAHS) can be defined as hemophagocytic syndrome occurring specifically in association with a flare or activity of underlying autoimmune disease. A recent study from Japan suggested that the incidence of AAHS is ∼10% among all cases of hemophagocytic syndrome ().
Autoimmune-associated Hemophagocytic Syndrome Kumakura, MD, PhD, Shunichi; Ishikura, MD, PhD, Hiroto; Umegae, MD, Nobuyuki; Yamagata, MD, Shingo; Kobayashi, MD, PhD, Shotai The reactive hemophagocytic syndrome is a benign histiocytic disorder characterized by hemophagocytosis by stimulated histiocytes in the bone marrow and reticuloendothelial systems, resulting in pancytopenia, liver dysfunction, and disseminated coagulopathy.