Hemophagocytic lymphohistiocytosis: An update on pathogenesis, diagnosis, and therapy

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening state of immune hyperactivation that arises in the setting of genetic mutations and infectious, inflammatory, or neoplastic triggers. Sustained, aberrant activation of cytotoxic CD8+ T cells and resultant inflammatory cytokine rele...

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Veröffentlicht in:	Best practice & research. Clinical rheumatology Jg. 34; H. 4; S. 101515
Hauptverfasser:	Griffin, Georgia, Shenoi, Susan, Hughes, Grant C.
Format:	Journal Article
Sprache:	Englisch
Veröffentlicht:	Netherlands Elsevier Ltd 01.08.2020
Schlagworte:	CD8-Positive T-Lymphocytes Cytokines Glucocorticoids Hemophagocytic lymphohistiocytosis (HLH) Humans Lymphohistiocytosis, Hemophagocytic - diagnosis Lymphohistiocytosis, Hemophagocytic - etiology Lymphohistiocytosis, Hemophagocytic - therapy Macrophage activation syndrome (MAS) Macrophage Activation Syndrome - diagnosis Macrophage Activation Syndrome - etiology Macrophage Activation Syndrome - therapy Hemophagocytic lymphohistiocytosis (HLH) Macrophage activation syndrome (MAS)
ISSN:	1521-6942, 1532-1770, 1532-1770
Online-Zugang:	Volltext
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Zusammenfassung:	Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening state of immune hyperactivation that arises in the setting of genetic mutations and infectious, inflammatory, or neoplastic triggers. Sustained, aberrant activation of cytotoxic CD8+ T cells and resultant inflammatory cytokine release are core pathogenic mechanisms. Key clinical features include high persistent fever, hepatosplenomegaly, blood cytopenia, elevated aminotransferase and ferritin levels, and coagulopathy. HLH is likely under-recognized, and mortality remains high, especially in adults; thus, prompt diagnosis and treatment are essential. Familial forms of HLH are currently treated with chemotherapy as a bridge to hematopoietic stem cell transplantation. HLH occurring in rheumatic disease (macrophage activation syndrome) is treated with glucocorticoids, IL-1 blockade, or cyclosporine A. In other forms of HLH, addressing the underlying trigger is essential. There remains a pressing need for more sensitive, context-specific diagnostic tools. Safer, more effective therapies will arise with improved understanding of the cellular and molecular mechanisms of HLH.
Bibliographie:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 ObjectType-Review-3 content type line 23
ISSN:	1521-6942 1532-1770 1532-1770
DOI:	10.1016/j.berh.2020.101515