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HLH-94 is a combination chemoimmunotherapy protocol developed by the Histiocyte Society in 1994 for the treatment of hemophagocytic lymphohistiocytosis (HLH), a life-threatening hyperinflammatory syndrome. The regimen combines chemotherapy and immunosuppressive agents, specifically etoposide (a topoisomerase II inhibitor), corticosteroids (such as dexamethasone), cyclosporin A (an immunosuppressant), and, in selected patients, intrathecal methotrexate. The protocol is used as induction therapy to control disease activity prior to hematopoietic stem cell transplantation in persistent or familial cases. It significantly improved survival rates for both primary and secondary forms of HLH compared to previous treatments[6][8].
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