Iranian Journal of Child Neurology

Vol. 1 No. 3 (2007)

GRISCELLI SYNDROME; A CASE REPORT AND REVIEW OF THE LITERATURE

Iranian Journal of Child Neurology, Vol. 1 No. 3 (2007), 17 January 2009 , Page 47-51
https://doi.org/10.22037/ijcn.v1i3.587

Abstract

Abstract:

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 activation.

We report a 5 months Old Iranian girl presenting with silver-gray hair,eyelashes and eyebrows, hepatosplenomegaly, pancytopenia, hemophagocytosis and progressive neurologic deterioration. Griscelli syndrome can be suggested according to her symptoms. The chemotherapy was not effective for her and she died due to multi organ failure.

Key words:

Griscelli syndrome, Hemophagocytosis, Albinism.

How to Cite

Shamsian MD, B., Arzanian MD, M., Alavi MD, S., & Zareifar MD, S. (2009). GRISCELLI SYNDROME; A CASE REPORT AND REVIEW OF THE LITERATURE. Iranian Journal of Child Neurology, 1(3), 47–51. https://doi.org/10.22037/ijcn.v1i3.587
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