Article
Treatment dilemmas in asymptomatic children with primary hemophagocytic lymphohistiocytosis.
Blood - 8 Nov 2018
Lucchini Giovanna, Marsh Rebecca, Gilmour Kimberly, Worth Austen, Nademi Zohreh, Rao Anupama, Booth Claire, Amrolia Persis, Silva Juliana, Chiesa Robert, Wynn Robert, Lehmberg Kai, Astigarraga Itziar, Güngör Tayfun, Stary Jan, Moshous Despina, Ifversen Marianne, Zinn Daniel, Jordan Michael, Kumar Ashish, Yasumi Takahiro, Veys Paul, Rao Kanchan
Abstract excerpt
Asymptomatic carriers (ACs) of pathogenic biallelic mutations in causative genes for primary hemophagocytic lymphohistiocytosis (HLH) are at high risk of developing life-threatening HLH, which requires allogeneic hematopoietic stem cell transplantation (HSCT) to be cured. There are no guidelines on the management of these asymptomatic patients. We analyzed the outcomes of pairs of index cases (ICs) and...
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