Stem cell transplantation for children with hemophagocytic lymphohistiocytosis: results from the HLH-2004 study

We report the largest prospective study thus far on hematopoietic stem cell transplantation (HSCT) in hemophagocytic lymphohistiocytosis (HLH), a life-threatening hyperinflammatory syndrome comprising familial/genetic HLH (FHL) and secondary HLH. Although all patients with HLH typically need intensi...

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Veröffentlicht in:	Blood advances 2020-08, Vol.4 (15), p.3754-3766
Hauptverfasser:	Bergsten, Elisabet, Horne, AnnaCarin, Hed Myrberg, Ida, Aricó, Maurizio, Astigarraga, Itziar, Ishii, Eiichi, Janka, Gritta, Ladisch, Stephan, Lehmberg, Kai, McClain, Kenneth L., Minkov, Milen, Nanduri, Vasanta, Rosso, Diego A., Sieni, Elena, Winiarski, Jacek, Henter, Jan-Inge
Format:	Artikel
Sprache:	eng
Schlagworte:	Adolescent Child Clinical Trials and Observations COVID-19 Hematopoietic Stem Cell Transplantation Humans Lymphohistiocytosis, Hemophagocytic - diagnosis Lymphohistiocytosis, Hemophagocytic - genetics Lymphohistiocytosis, Hemophagocytic - therapy Prospective Studies SARS-CoV-2 Systemic Inflammatory Response Syndrome
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Zusammenfassung:	We report the largest prospective study thus far on hematopoietic stem cell transplantation (HSCT) in hemophagocytic lymphohistiocytosis (HLH), a life-threatening hyperinflammatory syndrome comprising familial/genetic HLH (FHL) and secondary HLH. Although all patients with HLH typically need intensive anti-inflammatory therapy, patients with FHL also need HSCT to be cured. In the international HLH-2004 study, 187 children aged
ISSN:	2473-9529 2473-9537
DOI:	10.1182/bloodadvances.2020002101