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©2013 UpToDate ® Print Email Algorithm for the treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome HLH: hemophagocytic lymphohistiocytosis; MAS: macrophage activation syndrome; EBV: Epstein-Barr virus; CMV: cytomegalovirus; HIV: human immunodeficiency virus; JIA: juvenile idiopathic arthritis; HLH-94: HLH 1994 treatment protocol; CSA/Dex: treatment with cyclosporin + dexamethasone; XLP: X-linked lymphoproliferative syndrome; HCT: hematopoietic cell transplantation. * Check for mutations listed below. Since EBV, CMV, herpes, parvovirus, and many other viral infections can unmask HLH caused by one of these mutations, do NOT use presence of these infections to delay treatment with HLH-94. HCT is recommended for all patients <2 years of age, those with CNS-HLH, poor response to the first eight weeks of HLH-94 (or relapse), and all those with homozygous mutations of perforin, MUNC13-4, XLP, and syntaxin genes. • Griscelli & Chediak-Higashi syndromes, XLP, Kawasaki disease. Rashes of HLH may resemble Kawasaki-type rashes. HCT is needed for the first three of these diseases and for Kawasaki disease if poor response to HLH-94. Δ Solid organ or bone marrow transplantation. ◊ Bacterial or fungal infection, leishmaniasis, malaria. § Alemtuzumab should be employed for unresponsive HLH and may allow patients to undergo HCT [1] . Reference: Marsh RA, Allen CE, McClain KL, et al. Salvage therapy of refractory hemophagocytic lymphohistiocytosis with alemtuzumab. Pediatr Blood Cancer 2012.