This case report presents the management of a 25-year-old woman with refractory hemophagocytic lymphohistiocytosis (HLH) secondary to adult-onset Still's disease. Despite initial treatment with corticosteroids and biologic therapies, including anakinra and tocilizumab, her condition continued to deteriorate, necessitating intensive care unit admission. Following multidisciplinary consultation, ruxolitinib therapy, a Janus kinase (JAK) inhibitor targeting hyperinflammation, was initiated. Remarkably, the patient exhibited significant clinical improvement with resolution of fever, normalization of inflammatory markers, and organ function recovery. This case underscores the potential of ruxolitinib as a therapeutic option in refractory cases of HLH or macrophage activation syndrome, highlighting the importance of exploring novel therapies in refractory and challenging clinical scenarios.
Keywords: HLH; Hematology; immunology; oncology; ruxolitinib.
Ruxolitinib demonstrates promise as a therapeutic option in refractory cases of hemophagocytic lymphohistiocytosis (HLH).This case underscores the multisystem nature of HLH and macrophage activation syndrome, emphasizing the need for a comprehensive approach to diagnosis and management.Despite the availability of multiple treatment modalities, some cases of HLH may prove refractory to conventional therapies like anakinra and tocilizumab, emphasizing the need to consider different strategies and advance research about alternative therapies.
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