Breaking the storm: ruxolitinib as a lifeline in refractory hemophagocytic lymphohistiocytosis with macrophage activation syndrome.

Abstract

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.