[A hemophagocytic syndrome revealing a Griscelli syndrome type 2].

[oculocutaneous albinism]

Griscelli syndrome type 2 is a rare autosomal recessive disorder , due to a mutation in RAB 27 A gene . It associates partial albinism , silver hair and immune deficiency . We report the case of a 6 year -old boy who was admitted to the Emergency department with severe sepsis complicated by hemophagocytic syndrome . Many clinical and biological criteria leads to the diagnosis of type 2 Griscelli syndrome : consanguineous family , recurrent infection , absence of psychomotor retardation , oculocutaneous albinism , silver hair , occurrence of hemophagocytic syndrome and especially the pathognomonic appearance on microscopic examination of the hair . The absence of giant organelles inclusion in all granulated cells eliminated Chediak-Higashi syndrome .