Emapalumab

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Emapalumab
Monoclonal antibody
TypeWhole antibody
SourceHuman
TargetIFN-gamma
Clinical data
Pronunciation/ˈɛməpəlˌuməb/ EM-a-PAL-eu-mab[1]
Trade namesGamifant
Other namesNI-0501, emapalumab-lzsg
AHFS/Drugs.comMonograph
MedlinePlusa619024
License data
Routes of
administration
Intravenous
ATC code
Legal status
Legal status
Identifiers
CAS Number
DrugBank
ChemSpider
  • none
UNII
KEGG
E number{{#property:P628}}
CompTox Dashboard (EPA)
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Chemical and physical data
FormulaC6430H9898N1718O2038S46
Molar mass145352.66 g·mol−1

Emapalumab, sold under the brand name Gamifant, is an anti-interferon-gamma (IFNγ) antibody medication used for the treatment of hemophagocytic lymphohistiocytosis (HLH),[3][4][5] which has no cure.[6]

The most common side effects include infections, hypertension, infusion-related reactions, and pyrexia.[3]

The U.S. Food and Drug Administration (FDA) considers it to be a first-in-class medication.[7]

In June 2025, the U.S. Food and Drug Administration (FDA) approved emapalumab-lzsg for the treatment of macrophage activation syndrome (MAS) in patients with Still’s disease.[8]

Medical uses

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Emapalumab is used to treat primary hemophagocytic lymphohistiocytosis (HLH) with refractory, recurrent or progressive disease or intolerance with conventional HLH therapy.[3]

Adverse effects

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In the clinical trials that lead to emapalumab's FDA approval, the most commonly reported adverse effects were infections (56%), high blood pressure (41%), infusion reactions (27%), and fever (24%).[2][9] Serious adverse effects occurred in about half of the subjects studied in the clinical trial that led to its FDA approval.[9]

Pharmacology

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Mechanism of action

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In the setting of HLH, over-secretion of IFN-γ is thought to contribute to the pathogenesis of the disease.[2] Emapalumab binds and neutralizes IFN-γ, preventing it from inducing pathological effects.[2]

Pharmacokinetics

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Like other antibody-based medications, which are made of amino acid chains called polypeptides, emapalumab is broken down into smaller peptides via the body's normal catabolism.[2]

Society and culture

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The U.S. Food and Drug Administration (FDA) granted orphan drug designations in 2010 and 2020,[10][11] and breakthrough therapy designation in 2016, on the basis of preliminary data from the phase II trial.[12]

In July 2020, and again in November 2020, the European Medicines Agency (EMA) recommended the refusal of the marketing authorization for emapalumab.[13][14]

In June 2025, the FDA approved emapalumab-lzsg for the treatment of macrophage activation syndrome (MAS) in patients with Still’s disease.[8]

Research

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The research name of emapalumab was NI-0501.[1] A phase II/III trial began in 2013 and is ongoing as of August 2018.[15] The trial targets patients under the age of 18 who have failed to improve on conventional treatments.[16] This study was realised in the context of an EU-funded FP7 project, named FIGHT-HLH (306124).[citation needed]

References

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  1. ^ a b Lua error in Module:Citation/CS1/Configuration at line 2172: attempt to index field '?' (a nil value).
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  3. ^ a b c Lua error in Module:Citation/CS1/Configuration at line 2172: attempt to index field '?' (a nil value).[dead link] Public Domain This article incorporates text from this source, which is in the public domain.
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  5. ^ Lua error in Module:Citation/CS1/Configuration at line 2172: attempt to index field '?' (a nil value).
  6. ^ Clinical trial number NCT02069899 for "Long-term Follow-up of HLH Patients Who Received Treatment With NI-0501, an Anti-interferon Gamma Monoclonal Antibody" at ClinicalTrials.gov
  7. ^ Lua error in Module:Citation/CS1/Configuration at line 2172: attempt to index field '?' (a nil value).
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  15. ^ Clinical trial number NCT01818492 for "A Study to Investigate the Safety and Efficacy of an Anti-IFNγ mAb in Children Affected by Primary Haemophagocytic Lymphohistiocytosis" at ClinicalTrials.gov
  16. ^ Lua error in Module:Citation/CS1/Configuration at line 2172: attempt to index field '?' (a nil value).
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  • Clinical trial number NCT01818492 for "A Study to Investigate the Safety and Efficacy of an Anti-IFNγ mAb in Children Affected by Primary Haemophagocytic Lymphohistiocytosis" at ClinicalTrials.gov

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