Cerebral Involvement of Hemophagocytic Lymphohistiocytosis in Griscelli Syndrome
Journal Title: The Journal of Pediatric Research - Year 2019, Vol 6, Issue 3
Abstract
Type II Griscelli Syndrome (GS) is caused by a mutation in the RAB27A gene and usually manifests with silvery-gray hair, immune deficiency and the development of hemophagocytic lymphohistiocytosis (HLH). A hematopoietic stem cell transplantation is the curative treatment for HLH and reduced-intensity conditioning prevents the morbidity/mortality in the transplantation related to myeloablative conditioning. We report on a 21-month old boy with cerebral involvement of HLH related to GS.
Authors and Affiliations
Ersin Töret, Yılmaz Ay, Serap Aksoylar, Tuba Hilkay Karapınar, Yeşim Oymak
Keywords
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- EP ID EP656837
- DOI 10.4274/jpr.galenos.2018.33254
- Views 80
- Downloads 0
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