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KMID : 1084220240310020125
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Journal of Rheumatic Diseases
2024 Volume.31 No. 2 p.125 ~ p.129
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Successful treatment of hemophagocytic lymphohistiocytosis in a patient with systemic lupus erythematosus with ruxolitinib: a case report
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Jung Ji-In
Kim Ju-Yeon
Kim Mi-Hyeon
Park Jin-Kyun
Lee Eun-Young
Lee Eun-Bong
Park Jun-Won
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Abstract
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Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hematological disorder characterized by uncontrolled activation of CD8+ T and natural killer cells, leading to a cytokine storm and severe organ dysfunction. Although secondary HLH related to autoimmune diseases usually demonstrates a good treatment response to immunosuppressive therapy for underlying conditions, there is no consensus regarding the treatment in case of unresponsiveness to the treatment. Herein, we present a case of HLH that was unresponsive to high-dose glucocorticoid and cyclosporine treatment in a patient with newly diagnosed systemic lupus erythematosus. The patient’s clinical features and laboratory abnormalities rapidly improved with ruxolitinib, an oral Janus kinase 1 and 2 (JAK1/2) inhibitor. This result suggests that blocking JAK-STAT pathway may be a potential treatment option in patients with refractory HLH secondary to autoimmune diseases.
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KEYWORD
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Hemophagocytic lymphohistiocytosis, Janus kinase inhibitors, Ruxolitinib
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