Ruxolitinib as first-line therapy in secondary hemophagocytic lymphohistiocytosis and HIV infection

• Published in: International journal of hematology Vol. 112; no. 3; pp. 418 - 421
• Main Authors: Gálvez Acosta, Sergio, Javalera Rincón, Mariana
• Format: Journal Article
• Language: English
• Published: Singapore Springer Singapore 01-09-2020; Springer Nature B.V
• Subjects: Antigen-presenting cells; Antigens; Case Report; Chemotherapy; Complications; Cytokines; Enzyme inhibitors; Health services; Hematology; Histiocytosis; HIV; Human immunodeficiency virus; Immunological diseases; Infections; Inflammation; Inflammatory response; Inhibitor drugs; Janus kinase; Kinases; Lymphatic diseases; Lymphocytosis; Medical treatment; Medicine; Medicine & Public Health; Oncology; Targeted cancer therapy; Transfusion; Ruxolitinib; CMV/EBV infection; HIV infection; Secondary hemophagocytic lymphohistiocytosis
• ISSN: 0925-5710; 1865-3774
• DOI: 10.1007/s12185-020-02882-1

Secondary lymphohistiocytosis is a severe inflammatory condition characterized by uncontrolled inflammatory response hyperactivation of antigen-presenting cells, hemophagocytosis, cytopenias, and multiorgan failure, and is secondary to subjacent pathologies such as cancer, infection, or immune disease. Standard treatment includes chemotherapy; however, this is not always possible or safe. Ruxolitinib, a Janus kinase (JAK) 1/2 inhibitor can reduce inflammation and cytokine activity and has been used in refractory cases and first-line treatment. Here, we present a 35-year-old patient with HIV infection and secondary lymphohistiocytosis due to CMV/EBV infection who was successfully treated with ruxolitinib as first-line therapy. He rapidly showed clinical and biochemical improvement and transfusion independence without complications.