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Griscelli Syndrome with hemophagocytosis: a case report
International Journal of Contemporary Pediatrics
Griscelli syndrome is a rare autosomal recessive disorder characterized by pigmentary dilution of the skin and the hair (silver hair), the presence of large clumps of pigment in hair shafts, and an accumulation of melanosomes in melanocytes. In one variant, hepatosplenomegaly, lymphohistiocytosis, and a combined T-cell and B-cell immunodeficiency are pronounced. The associated immunodeficiency often involves impaired natural killer cell activity, absent delayed-type hypersensitivity, and a poordoi:10.18203/2349-3291.ijcp20162408 fatcat:wty2kbzmorfyji2afmkginlhxu