GRISCELLI SYNDROME; A CASE REPORT AND REVIEW OF THE LITERATURE
نویسندگان
1 Assistant professor, Department of pediatric hematology-oncology,Mofid children's hospital,Shaid BEheshti Medical University
2 Assistant professor ,Cheif of hematology-oncology ward, Department of pediatric hematology-oncology, Shaid Beheshti Medical University
3 Assistant professor, Department of pediatric hematology-oncology, Namazi Hospital, Shiraz University of medical sciences
4 Assistant professor, Department of pediatric hematology-oncology, Namazi Hospital, Shiraz University of medical sciences
doi
10.22037/ijcn.v1i3.587چکیده
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.
