Figure 1.

Pathogenesis of hemophagocytic lymphohistiocytosis. The function of LYST, probably important for correct size and function of lytic granules, is not entirely understood. Note the empty granula in perforin deficiency. Adapted from [8,47,88]. CHS, Chédiak Higashi syndrome; CTL, CD8+ cytotoxic T lymphocyte; FHL, familial hemophagocytic lymphohistiocytosis; GSII, Griscelli syndrome type II; HPSII, Hermansky-Pudlak syndrome type II; NK, natural killer.