Indication
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.
Important Safety Information
Infections
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.
Indication
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.
Important Safety Information
Infections
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.
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, 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.
Adverse Reactions
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.
Click here for full Prescribing Information for Gamifant.
You may also contact Sobi at medinfo.us@sobi.com or 866-773-5274.
References
- Gamifant (emapalumab-lszg) prescribing information. Stockholm, Sweden: Sobi, Inc. 2022.
- 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
- Data on file. Stockholm, Sweden: Swedish Orphan Biovitrum AB. 2018.
- 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
- 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
- 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
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.
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