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INDICATIONS

Gamifant (emapalumab-lzsg) is an interferon gamma (IFNγ)-neutralizing 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.
  • HLH/macrophage activation syndrome (MAS) in known or suspected Still’s disease, including systemic Juvenile Idiopathic Arthritis (sJIA), with an inadequate response or intolerance to glucocorticoids, or with recurrent MAS.
IMPORTANT SAFETY INFORMATION
Infections

Gamifant may increase the risk of fatal and serious infections with pathogens including mycobacteria, herpes zoster virus... and histoplasma capsulatum. Do not administer Gamifant in patients with these infections until appropriate treatment has been initiated.

INDICATIONS

Gamifant (emapalumab-lzsg) is an interferon gamma (IFNγ)-neutralizing 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.
  • HLH/macrophage activation syndrome (MAS) in known or suspected Still’s disease, including systemic Juvenile Idiopathic Arthritis (sJIA), with an inadequate response or intolerance to glucocorticoids, or with recurrent MAS.

IMPORTANT SAFETY INFORMATION

Infections

Gamifant may increase the risk of fatal and serious infections with pathogens including mycobacteria, herpes zoster virus, and histoplasma capsulatum. Do not administer Gamifant in patients with these infections until appropriate treatment has been initiated.

In patients with primary HLH receiving Gamifant in clinical trials, serious infections such as sepsis, pneumonia, bacteremia, disseminated histoplasmosis, necrotizing fasciitis, viral infections, and perforated appendicitis were observed in 32% of patients.

In patients with HLH/MAS in Still’s disease receiving Gamifant in clinical trials, serious infections such as pneumonia, cytomegalovirus infection, cytomegalovirus infection reactivation, and sepsis were observed in 13% of patients.

Evaluate patients for tuberculosis risk factors and test for latent infection prior to initiating Gamifant. Administer tuberculosis prophylaxis to patients at risk for tuberculosis or known to have a positive purified protein derivative (PPD) test result.

Consider prophylaxis for herpes zoster, Pneumocystis jirovecii, and fungal infection while receiving Gamifant. Employ surveillance testing during treatment with Gamifant.

Closely monitor patients receiving Gamifant for signs or symptoms of infection, promptly initiate a complete diagnostic workup appropriate for an immunocompromised patient, and initiate appropriate antimicrobial therapy.

Increased Risk of Infection With Use of Live Vaccines

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

Infusion-related reactions in patients with primary HLH, 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.

Infusion-related reactions in patients with HLH/MAS in Still’s disease, including pyrexia, headache, paresthesia, bone pain, pruritic rash, and peripheral coldness, were reported with Gamifant treatment in 13% of patients. Infusion-related reactions were reported as mild in 8% of patients and as moderate in 5% of patients.

Monitor patients for infusion-related reactions, which can be severe. Interrupt the infusion for infusion reactions and institute appropriate medical management before continuing infusion at a slower rate.

Adverse Reactions

Primary HLH

Serious adverse reactions were reported in 53% of patients. The most common serious adverse reactions (≥3%) included infections, gastrointestinal hemorrhage, and multiple organ dysfunction. Fatal adverse reactions occurred in 2 (6%) of patients and included septic shock and gastrointestinal hemorrhage.

The most common adverse reactions were (≥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%).

HLH/MAS

Serious adverse reactions were reported in 12 patients (31%), with the most common serious adverse reaction being pneumonia (5%). Fatal adverse reactions occurred in two patients (5%) and included multiple organ dysfunction and circulatory shock.

The most common adverse reactions (≥10%) for Gamifant included viral infection (44%), rash (21%), anemia (18%), leukopenia (15%), thrombosis (15%), bacterial infections (13%), headache (13%), hyperglycemia (13%), infusion-related reactions (13%), abdominal pain (10%), hypertension (10%), pyrexia (10%), and thrombocytopenia (10%).

References

  1. Gamifant (emapalumab-lszg) prescribing information. Stockholm, Sweden: Sobi, Inc. 2022.
  2. Locatelli F, Jordan MB, Allen C, et al. Emapalumab in children with primary hemophagocytic lymphohistiocytosis. N Engl J Med. 2020;382(19):1811-1822. doi:10.1056/NEJMoa1911326
  3. Data on file. Stockholm, Sweden: Swedish Orphan Biovitrum AB. 2018.
  4. De Benedetti F, Prencipe G, Bracaglia C, Marasco E, Grom AA. Targeting interferon-γ in hyperinflammation: opportunities and challenges. Nat Rev Rheumatol. 2021;17(11):678-691. doi:10.1038/s41584-021-00694-z
  5. Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL. How I treat hemophagocytic lymphohistiocytosis. Blood. 2011;118(15):4041-4052. doi:10.1182/blood-2011-03-278127
  6. Sepulveda FE, de Saint Basile G. Hemophagocytic syndrome: primary forms and predisposing conditions. Curr Opin Immunol. 2017;49:20-26. doi:10.1016/j.coi.2017.08.004

Gamifant® (emapalumab-lzsg) was proven to be effective in a clinical trial1

For patients with refractory, recurrent, or progressive disease or intolerance to conventional hemophagocytic lymphohistiocytosis (HLH) therapy.1

Young boy crouched with arms crossed over his knees

63% (17/27) overall response rate (ORR) with Gamifant1

(95% CI: 0.42, 0.81; P = 0.013)

70% of patients (19/27) proceeded to HSCT1

HSCT=hematopoietic stem cell transplantation.

  • ORR is defined as achievement of either complete or partial response in HLH improvement1
  • Median time to response was 8 days2
  • Durability of first response was maintained for 18 days in 75% of the previously treated patients and for 26 days in 75% of the patients who received Gamifant2
  • HSCT=hematopoietic stem cell transplantation.
CXCL9 concentration graph

Measuring CXCL9 concentration was a prognostic factor for clinical response in the Gamifant pivotal trial. It is stable and easy to measure in serum at nanogram concentrations.3,4

Gamifant was shown to neutralize IFNγ3

Gamifant was shown to neutralize interferon gamma (IFNγ) as measured by a rapid and sustained reduction in the plasma concentrations of CXCL9, a chemokine induced almost exclusively by IFNγ. This is consistent with the early onset of clinical action and time to response seen with Gamifant.3

  • The median time to first response to treatment was 8 days2
  • An 82% median reduction from baseline was observed at day 14 of Gamifant treatment3
  • Reductions in plasma concentrations of CXCL9 were sustained throughout the study3
See how Gamifant works

See The Real-World Evidence