原发性原发性HPS,或称家族性HPS,为常染色体隐性遗传病,其发病和病情加剧常与感染有关。继发性继发性HPS分为感染相关性HPS(infection-associatedhemophagocyticsyndrome,IAHS),此型多与病毒感染有关,由病毒引起者称病毒相关性HPS(virus-associatedhemophagocyticsyndrome,VAHS);由肿瘤引起者称肿瘤相关性HPS(malignancy-associatedhemophagnocyticsyndrome,MAHS)。