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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

  1. Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL. How I treat hemophagocytic lymphohistiocytosis. Blood. 2011;118(15):4041-4052. doi: https://doi.org/10.1182/blood-2011-03-278127.
  2. Morimoto A, Nakazawa Y, Ishii E. Hemophagocytic lymphohistiocytosis: pathogenesis, diagnosis, and management. Pediatr Int. 2016;58(9):817-825.
  3. George M. Hemophagocytic lymphohistiocytosis: review of etiologies and management. J Blood Med. 2014;5:69-86. http://dx.doi.org/10.2147/JBM.S46255.
  4. Bergsten E, Horne A, Arico M, et al. Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study. Blood. 2017;130:2728-2738. doi:10.1182/blood-2017-06-788349.
  5. Marsh RA, Haddad E. How I treat primary haemophagocytic lymphohistiocytosis. Br J Haematol. 2018;182(2):185-199. doi: 10.1111/bjh.15274.
  6. Etopophos [prescribing information]. Deerfield, IL: Baxter Healthcare Corporation; 2017.
  7. Bode SFN, Lehmberg K, Maul-Pavicic A, et al. Recent advances in the diagnosis and treatment of hemophagocytic lymphohistiocytosis. Arthritis Res Ther. 2012;14(3)213. doi:10.1186/ar3843.
  8. Gamifant [prescribing information]. Stockholm, Sweden: Swedish Orphan Biovitrum AB.
  9. FDA approves first treatment specifically for patients with rare and life-threatening type of immune disease [news release]. Silver Spring, MD: Food and Drug Administration; November 20, 2018. https://www.fda.gov/newsevents/newsroom/pressannouncements/ucm626263.htm. Accessed July 21, 2020.
  10. Price B, Lines J, Lewis D, Holland N. Haemphagocytic lymphohistiocytosis: a fulminant syndrome associate with multiorgan failure and high mortality that frequently masquerades as sepsis and shock. S Afr Med J. 2014; 104(6):401-406. doi:10.7196/samj.7810.
  11. Sepulveda F, de Saint Basile G. Hemophagocytic syndrome: primary forms and predisposing conditions. Curr Opin Immunol. 2017; 49:20-26. http://dx.doi.org/10.1016/j.coi.2017.08.004.
  12. Lehmberg K, Nichols KE, Henter J-I, et al. Consensus recommendations for the diagnosis and management of hemophagocytic lymphohistiocytosis associated with malignancies. Haematologica. 2015:100(8):997-1004.

Getting patients to transplant

For patients with primary hemophagocytic lymphohistiocytosis (HLH), a successful hematopoietic stem cell transplantation (HSCT) is the ultimate goal. Meanwhile, keeping inflammatory symptoms under control with the proper treatment is crucial.1-3

Treatment has 2 main goals:

Subdue the cytokine storm

Immediately bring the cytokine storm under control to prevent irreversible organ damage. Subsequent treatment depends on symptom presentation and confirmation of diagnosis.1-3

Condition for transplant

Condition the patient for HSCT in a way that optimizes their long-term prognosis.1

Data show that 50% of patients fail to reach HSCT due to inadequate response to conventional treatments.4

The treatment landscape

Over the past 25 years, few advancements have been made in targeted HLH therapy.5

Conventional treatments—which were not specifically developed for primary HLH and which are not FDA approved for the condition—are aimed at immune suppression.1,6

When a patient responds unsatisfactorily to conventional treatment, cannot tolerate it, or relapses, there has been no standard alternative. HSCT is always the goal, but physicians may have little choice but to rush poorly conditioned patients into the procedure, or forgo transplant altogether.1,5,7

There has been a clear need for a targeted option with an established safety profile to treat patients with primary HLH.1

A targeted Treatment

Gamifant® (emapalumab-lzsg) is the first and only treatment designed specifically for primary HLH. It works by targeting IFNγ, a central and upstream cytokine in the pathogenesis of the disease.1-3

See how it works