Survival and EFS. (A) OS; 3-year pSU for patients with pHLH in the cohort including patients who were asymptomatic: FHL (n = 63) vs hereditary non-FHL (n = 25). (B) Three-year pSU for patients who were symptomatic and received first-line etoposide (n = 57) or other major drug(s) (n = 9). (C) Three-year EFS for all patients with pHLH including patients who were asymptomatic. (D) Three-year EFS for patients who were symptomatic and received etoposide or other major drug(s). In panels A and C, the blue line represents patients with FHL; red line represents patients with hereditary non-FHL. In panels B and D, the green line represents first-line treatment with etoposide and the purple line represents first-line treatment with other major drugs. (E) Overall 3-year pSU of patients who were symptomatic (n = 76; orange line) vs asymptomatic (n = 12; dark turquoise line) at the time of diagnosis. (F) Post-HSCT 3-year pSU of patients who were symptomatic treated with first-line etoposide (n = 52; green line).