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More than half of patients suffered from CNS involvement, an unfavorable prognostic factor.5 Strikingly, the here-reported patient showed progressive central HLH while under immunosuppressive therapy, and additional treatment with ATG had no effect. Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory condition caused by excessive release of pro-inflammatory cytokines, typically resulting in fever, cytopenia, and hepatosplenomegaly.1 HLH can be life-threatening due to rapid progression to multi-organ failure. Keywords: cholestasis; familial hemophagocytic lymphohistiocytosis; hemophagocytic lymphohistiocytosis; Munc13-4; neonatal; UNC13D EN cholestasis familial hemophagocytic lymphohistiocytosis hemophagocytic lymphohistiocytosis Munc13-4 neonatal UNC13D 1 4 4 04/28/22 20220401 NES 220401 CONFLICT OF INTEREST None of the authors has any potential conflict of interest related to this manuscript. However, in the first year of life, primary or genetic forms of HLH are more prevalent than secondary forms, and a complete diagnostic workup should always be performed.1 In this case, screening for primary HLH was delayed until the second HLH episode at 4 months of age, and the genetic diagnosis of FHL3 was only established at the age of 7 months. [Extracted from the article]