Ulus Travma Acil Cerrahi Derg. 2019; 6(3): 252-255 | |||
Cerebral Involvement of Hemophagocytic Lymphohistiocytosis in Griscelli SyndromeErsin Töret1, Yılmaz Ay1, Serap Aksoylar2, Tuba Hilkay Karapınar1, Yeşim Oymak11Çocuk Hematoloji Onkoloji Kliniği, Dr. Behcet Uz Çocuk Sağlığı Ve Cerr. Hast., Izmir, Turkey2Çocuk 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 TransplantationErsin Töret, Yılmaz Ay, Serap Aksoylar, Tuba Hilkay Karapınar, Yeşim Oymak. Cerebral Involvement of Hemophagocytic Lymphohistiocytosis in Griscelli Syndrome. Ulus Travma Acil Cerrahi Derg. 2019; 6(3): 252-255 Corresponding Author: Ersin Töret, Türkiye |
|