The Journal of Pediatric Research

Cerebral Involvement of Hemophagocytic Lymphohistiocytosis in Griscelli Syndrome []
. 2019; 6(3): 0-0

Cerebral Involvement of Hemophagocytic Lymphohistiocytosis in Griscelli Syndrome

Ersin Töret1, Yılmaz Ay1, Serap Aksoylar2, Tuba Hilkay Karapınar1, Yeşim Oymak1
1Çocuk Hematoloji Onkoloji Kliniği, Dr. Behcet Uz Çocuk Sağlığı Ve Cerr. Hast., Izmir, Turkey
2Çocuk Hematoloji Onkoloji Bd - Pediatrik Ki̇t Ünitesi, Izmir, Turkey

Type 2 Griscelli Syndrome is caused by a mutation in RAB27A gene and usually manifested with silvery-gray hair, immune deficiency and development of hemophagocytic lymphohistiocytosis. Hematopoetic stem cell transplantation is the curative treatment for HLH and reduced-intensity conditioning prevented the morbidity/mortality in the transplantation related to the myeloablative conditioning. We reported a 21 months old boy with cerebral involvement of HLH related to GS.

Keywords: Griscelli Syndrome, Hemophagocytic Lymphohistiocytosis, Hematopoetic Stem Cell Transplantation


Ersin Töret, Yılmaz Ay, Serap Aksoylar, Tuba Hilkay Karapınar, Yeşim Oymak. Cerebral Involvement of Hemophagocytic Lymphohistiocytosis in Griscelli Syndrome. . 2019; 6(3): 0-0

Corresponding Author: Ersin Töret, Türkiye


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