Clofarabine is a purine nucleoside which has demonstrated efficacy in the treatment of relapsed leukemia in children. Among its adverse effects has been described the systemic inflammatory response syndrome development. This rare and potentially serious syndrome is due to the deregulation of inflammatory response. We described a case of a 10-year-old patient, with second relapse of acute lymphoblastic leukemia precursor B-cell, who developed a severe systemic inflammatory response syndrome during the administration of combined treatment of clofarabine, etoposide, and cyclophosphamid resulting into death. The causal relation between the adverse reaction and clofarabine administration was established as probable by the application of modified Karch-Lasagna algorithm
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