Successful treatment of fatal macrophage activation syndrome and haemophagocytic lymphohistiocytosis by combination therapy including continuous haemodiafiltration with a cytokine-adsorbing haemofilter (AN69ST) in a patient with systemic lupus erythematosus

Abstract

Continuous haemodiafiltration (CHDF) with a cytokine-adsorbing haemofilter (AN69ST–CHDF) was successfully used to treat systemic lupus erythematosus (SLE) complicated with macrophage activation syndrome (MAS) and haemophagocytic lymphohistiocytosis (HLH) in a 35-year-old male. Five months before admission, serositis, proteinuria, and neuropsychiatric SLE recurred. He was treated with glucocorticoid, rituximab, and intravenous cyclophosphamide. He was subsequently admitted for the examination of hepatic disorder and leukopenia. Following admission, he was diagnosed with MAS/HLH, and glucocorticoid pulse therapy was initiated. Although leukopenia temporarily improved, his pancytopenia and hepatic dysfunction worsened, leading to multiple organ dysfunction syndrome (MODS) on day 22. Because hypercytokinemia contributed to MAS/HLH exacerbation, we implemented a combination therapy with cyclosporine A and plasma exchange. This strategy failed to improve MODS; thus, AN69ST–CHDF was initiated and added rituximab which led to improved hypercytokinemia and eventual recovery from MODS. AN69ST–CHDF might be an effective therapeutic option for MAS/HLH.

Keywords:

• Systemic lupus erythematosus
• haemophagocytic lymphohistiocytosis
• macrophage activation syndrome
• continuous haemodiafiltration with a cytokine-adsorbing haemofilter

Patient consent

Written informed consent for publication was obtained from the patient.

Conflict of interest

None.