LYMPHOHISTIOCYTOSIS

We report a case, a 2 months old male child who presented with fever, letharginess, abdominal distension having silvery gray hairs of eye brow, eyelash scalp. Clinical features investigations revealed hemophagocytic lymphohistiocytosis. Hair shaft examination revealed irregular large clumps of melanin pigment aggregations. He was diagnosed as a case of Type 2 Griscelli Syndrome with Hemophagocytic Lymphohistiocytosis. Griscelli syndrome is a rare inherited disorder that was originally described in 1978. So far, about 90 cases have been reported worldwide.