Abstract
Hemophagocytic lymphopcytosis (HLH) is a life-threatening condition. It can occur either as primary form with genetic defects or secondary to other conditions, such as hematological or autoimmune diseases. Certain triggering factors can predispose individuals to the development of HLH. We report the case of a 25-year-old male patient who was diagnosed with HLH in the context of adult-onset Still's disease (AOSD) during a primary infection with Epstein-Barr virus (EBV). During therapy with anakinra and dexamethasone, he was still symptomatic with high-spiking fevers, arthralgia, and sore throat. His laboratory values showed high levels of ferritin and C-reactive protein. His condition improved after the addition of rituximab and cyclosporine to his immunosuppressive regimen with prednisolone and anakinra. This combination therapy led to a sustained clinical and serological remission of his condition. While rituximab has been used successfully for HLH in the context of EBV-associated lymphoma, its use in autoimmune diseases is uncommon. We hypothesize that the development of HLH was triggered by a primary EBV infection and that rituximab led to elimination of EBV-infected B-cells, while cyclosporine ameliorated the cytokine excess. We therefore propose that this combination immunosuppressive therapy might be successfully used in HLH occurring in the context of autoimmune diseases.
Highlights
Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening condition characterized by excessive macrophage activity and cytokine production resulting in multiorgan failure [1, 2]
Primary HLH occurs in children with HLH-associated genetic defects or a family history of HLH, while secondary HLH develops after exposure to immunological trigger factors of underlying infections, autoimmune diseases, or malignancies [3]
We report the case of a 25-year-old man who had been diagnosed with adult-onset Still’s disease (AOSD) five years before admission
Summary
Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening condition characterized by excessive macrophage activity and cytokine production resulting in multiorgan failure [1, 2]. Primary HLH occurs in children with HLH-associated genetic defects or a family history of HLH, while secondary HLH develops after exposure to immunological trigger factors of underlying infections, autoimmune diseases, or malignancies [3]. In the setting of rheumatic conditions, HLH is termed macrophage activation syndrome (MAS) and occurs most frequently in patients with systemic juvenile idiopathic arthritis (sJIA), adult-onset Still’s disease (AOSD), and systemic lupus erythematosus (SLE) [4]
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