Cell versus cytokine – directed therapies for hemophagocytic lymphohistiocytosis (HLH) in inborn errors of immunity
Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a heterogeneous hyperinflammatory syndrome with different pathways of pathogenesis resulting in similar clinical presentations. It is best defined and understood if presenting in the context of genetic immunodeficiencies associated with defects of lymphocyte cytotoxicity. In these “primary” forms of HLH, cellular and soluble immune effectors are relatively well characterized. While etoposide-based broad cell-directed therapies remain standard of care, more specific therapies targeting these effectors individually are increasingly available. Anti-CD52 as a cell-directed therapy and anti-IFN-gamma, IL-18BP, and JAK-inhibition as cytokine-directed therapies are expected to broaden the therapeutic options, but the precise role of these drugs in first-line and rescue treatment indications remains to be defined. A number of additional inborn errors of immunity are associated with episodes of immune activation fulfilling the clinical criteria of HLH. Impaired pathogen control is a key driver of hyperinflammation in some conditions, while others are characterized by a strong autoinflammatory component. This heterogeneity of disease-driving factors and the variable severity in disease progression in these conditions do not allow a simple adaptation of protocols established for “primary” HLH to HLH in the context of other inborn errors of immunity. Cytokine-directed therapies hold significant promise in these increasingly recognized disorders
- Location
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Deutsche Nationalbibliothek Frankfurt am Main
- Extent
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Online-Ressource
- Language
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Englisch
- Notes
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Frontiers in immunology. - 11 (2020) , 808, ISSN: 1664-3224
- Keyword
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Hämophagozytische Lymphohistiozytose
Pathogenese
Immunität
Therapie
Cytokine
Entzündung
- Event
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Veröffentlichung
- (where)
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Freiburg
- (who)
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Universität
- (when)
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2020
- Creator
- DOI
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10.3389/fimmu.2020.00808
- URN
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urn:nbn:de:bsz:25-freidok-1658602
- Rights
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Der Zugriff auf das Objekt ist unbeschränkt möglich.
- Last update
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14.08.2025, 10:50 AM CEST
Data provider
Deutsche Nationalbibliothek. If you have any questions about the object, please contact the data provider.
Associated
- Wegehaupt, Oliver Philipp
- Wustrau, Katharina
- Lehmberg, Kai
- Ehl, Stephan
- Universität
Time of origin
- 2020
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Graded defects in cytotoxicity determine severity of hemophagocytic lymphohistiocytosis in humans and mice
Immune‐mediated pathology as a consequence of impaired immune reactions: the IMPATH paradox
Trigger‐dependent differences determine therapeutic outcome in murine primary hemophagocytic lymphohistiocytosis
Your critical care patient may have HLH (hemophagocytic lymphohistiocytosis)
Trigger‐dependent differences determine therapeutic outcome in murine primary hemophagocytic lymphohistiocytosis
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Autoimmune lymphoproliferative immunodeficiencies (ALPID) in childhood: breakdown of immune homeostasis and immune dysregulation
Immune‐mediated pathology as a consequence of impaired immune reactions: the IMPATH paradox
Adoptive T cell therapy cures mice from active hemophagocytic lymphohistiocytosis (HLH)
Adoptive T cell therapy cures mice from active hemophagocytic lymphohistiocytosis (HLH)
Disease pathophysiology in murine models of primary hemophagocytic lymphohistiocytosis: triggers and effectors
Graded defects in cytotoxicity determine severity of hemophagocytic lymphohistiocytosis in humans and mice
Immune‐mediated pathology as a consequence of impaired immune reactions: the IMPATH paradox
Trigger‐dependent differences determine therapeutic outcome in murine primary hemophagocytic lymphohistiocytosis
Your critical care patient may have HLH (hemophagocytic lymphohistiocytosis)
Trigger‐dependent differences determine therapeutic outcome in murine primary hemophagocytic lymphohistiocytosis
The microbiome modifies manifestations of hemophagocytic lymphohistiocytosis in perforin‐deficient mice
GATA2 deficiency and hemophagocytic lymphohistiocytosis (HLH): a systematic review of reported cases
Autoimmune lymphoproliferative immunodeficiencies (ALPID) in childhood: breakdown of immune homeostasis and immune dysregulation