Abstract
BackgroundAutoimmune disease related hemophagocytic syndrome, in other words, macrophage activation syndrome(MAS), is a rare, but lethal complication of autoimmune disease. At present, specific treatment guidelines for adult MAS have not been formulated, most experience are derived from children, researches about etoposide are scarce. As the importance of etoposide in the initial treatment had been proved in other subtypes of hemophagocytic syndrome, the objective of this study is to investigate the effectiveness of etoposide in the treatment of the adult macrophage activation syndrome.Result74 patients with autoimmune disease related hemophagocytic syndrome were involved in this study, they were divided into two groups based on initial treatment, group 1(n=53): initial therapy did not contain etoposide, group 2(n=21): initial therapy contained etoposide. The overall response rate and complete response rate of group 2 were significantly higher than group 1(ORR 90.5% vs 24.5%, CRR 33.3% vs 3.8%, P<0.05). Patients with different HLH remission states have significantly different prognosis(P<0.001).ConclusionAdopting VP-16 in initial treatment can significantly increase the OR rate and CR rate of adult MAS patients, and the HLH states influenced the prognosis significantly.
Autoimmune-Associated Hemophagocytic Syndrome/Macrophage Activation Syndrome