IFNγ in primary HLH
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Gamifant® (emapalumab-lzsg) is an interferon gamma (IFNγ)–blocking antibody indicated for the treatment of adult and pediatric (newborn and older) patients with primary... hemophagocytic lymphohistiocytosis (HLH) with refractory, recurrent, or progressive disease or intolerance with conventional HLH therapy.
Before initiating Gamifant, patients should be evaluated for infection, including latent tuberculosis (TB)... Prophylaxis for TB should be administered to patients who are at risk for TB or known to have a positive purified protein derivative (PPD) test result or positive IFNγ release assay.
Gamifant® (emapalumab-lzsg) is an interferon gamma (IFNγ)–blocking antibody indicated for the treatment of adult and pediatric (newborn and older) patients with primary hemophagocytic lymphohistiocytosis (HLH) with refractory, recurrent, or progressive disease or intolerance with conventional HLH therapy.
Before initiating Gamifant, patients should be evaluated for infection, including latent tuberculosis (TB). Prophylaxis for TB should be administered to patients who are at risk for TB or known to have a positive purified protein derivative (PPD) test result or positive IFNγ release assay.
During Gamifant treatment, patients should be monitored for TB, adenovirus, Epstein-Barr virus (EBV), and cytomegalovirus (CMV) every 2 weeks and as clinically indicated.
Patients should be administered prophylaxis for herpes zoster, Pneumocystis jirovecii, and fungal infections prior to Gamifant administration.
Do not administer live or live attenuated vaccines to patients receiving Gamifant and for at least 4 weeks after the last dose of Gamifant. The safety of immunization with live vaccines during or following Gamifant therapy has not been studied.
Infusion-related reactions, including drug eruption, pyrexia, rash, erythema, and hyperhidrosis, were reported with Gamifant treatment in 27% of patients. In one-third of these patients, the infusion-related reaction occurred during the first infusion.
In the pivotal trial, the most commonly reported adverse reactions (≥10%) for Gamifant included infection (56%), hypertension (41%), infusion-related reactions (27%), pyrexia (24%), hypokalemia (15%), constipation (15%), rash (12%), abdominal pain (12%), CMV infection (12%), diarrhea (12%), lymphocytosis (12%), cough (12%), irritability (12%), tachycardia (12%), and tachypnea (12%).
Additional selected adverse reactions (all grades) that were reported in less than 10% of patients treated with Gamifant included vomiting, acute kidney injury, asthenia, bradycardia, dyspnea, gastrointestinal hemorrhage, epistaxis, and peripheral edema.
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Interferon gamma (IFNγ) is the only type II interferon and plays an important role in cell communication during immune responses. During innate immune responses, IFNγ helps eliminate intracellular pathogens by activating macrophages and natural killer (NK) cells. During adaptive immune responses, IFNγ is responsible for both the differentiation and overproliferation of activated T cells.1,2
In primary hemophagocytic lymphohistiocytosis (HLH), the immune system is dysregulated—and IFNγ contributes directly to disease pathogenesis.1,3
In healthy individuals, antigen-presenting cells (APCs) are recognized by cytotoxic CD8+ T cells, which bind to them to release perforin and granzymes into the immunological synapse space. Perforin creates pores in the target cell's plasma membrane, allowing the cytotoxic granzymes to enter and initiate lysis. In primary HLH, genetic mutations prevent perforin pore formation needed for cell lysis.4-6
Click through to see how IFNγ-activated macrophages trigger the downstream release of proinflammatory cytokines—including additional IFNγ—perpetuating hypercytokinemia and hyperinflammation in an aggressive continuum.1
IFNγ drives the uncontrolled release of cytokines, which result in the rapidly progressive and life-threatening symptoms of primary HLH.
For more information about the critical role of IFNγ in primary HLH, download this guide.
IFNγ GuideSee how IFNγ triggers clinical and laboratory manifestations of disease.
IFNγ was found to be essential for the development of HLH-like pathology. In murine models, inhibition of this cytokine led to an improvement of known features of HLH, including2,3: