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Griscelli syndrome (GS) type II is a rare autosomal recessive disorder characterized by.partial albinism along with, hemophagocytic lymphohistiocytosis (HLH). There are characteristic light microscopy findings in scalp hair shaft seen as large irregular clumps of pigment. An 8 month old boy presented with fever for 15 days, and swollen lower legs since 10 days. He was the 6th child of second degree consanguineous marriage. He had silvery white hair (A). He had recurrent episodes of fever since 3 months of age. On examination he had fever, jaundice and pitting edema of lower limbs (B).  Liver and spleen were enlarged. WBC was 4 X10 9/L, Hb: 9 gm/dL, Platelets: 8 X 10 9/L. He had hypertriglyceridemia ( 358  mg/dL) ,hypofibrinogenmia (96 mg/dL) and hyperferritinemia ( 1458 ng/ml ). Bone marrow aspirate did not show increased hemophagocytosis.  He had partial albinism and silvery white hair of eyebrows, scalp hair and eyelashes . Light microscopy of scalp hair shaft was performed, which showed characteristic large irregular clumps of pigment suggesting GS-II . Griscelli Syndrome should be suspected when there is characteristic phenotypic appearance of partial albinism associated with HLH.