Dr Ananda Kumar
Abstract: Griscelli syndrome is an rare autosomal recessive disorder characterized by Partial albinism with variable immunodeficiency. Silvery gray hair with large clumped melanasomes on microscopy of hair shafts are diagnostic. The commonest complication leading to mortality includes lymphohistiocytic proliferation in various organs, including brain.
Keywords: griscelli syndrome, hemophagocytosis, Lymphohistiocytic proliferation, Silvery gray hair, hepatosplenomegaly