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GRISCELLI SYNDROME; A CASE REPORT AND REVIEW OF THE LITERATURE

SHAMSIAN B. SH. نويسنده , Arzanian M. T. نويسنده , ALAVI S. نويسنده , ZEREIFAR S. نويسنده

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Griscelli syndrome (GS) is a rare disease first described in 1978. It is inherited in autosomal recessive pattern. This disease is characterized by partial albinism, pigmentation dilution, cellular immunodeficiency, neurological involvement & uncontrolled phases of macrophage & lymphocyte activati on. We report a 5 months Old Iranian girl presenting with silver-gray hair, eyelashes and eyebrows, hepatosplenomegaly, pan cytopenia, hemophagocytosis and progressive neurol ogic deterioration. Griscelli synd rome can be suggested acco rd ing to her symptoms. The chemotherapy was not effective for her and she died due to multi organ failure.

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