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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.

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. Gamifant [prescribing information]. Stockholm, Sweden: Swedish Orphan Biovitrum AB.
  2. 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.
  3. 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.
  4. 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.
  5. 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.
  6. Locatelli F, Jordan MB, Allen C, et al. Emapalumab in children with primary hemophagocytic lymphohistiocytosis. N Engl J Med. 2020;382(19):1811-1822.
  7. Jordan M, Hildeman D, Kappler J, Marrack P. An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorder. Blood. 2004;104(3): 735-743. doi: https://doi.org/10.1182/blood-2003-10-3413.
  8. Morimoto A, Nakazawa Y, Ishii E. Hemophagocytic lymphohistiocytosis: pathogenesis, diagnosis, and management. Pediatr Int. 2016;58(9):817-825.
  9. Bracaglia C, Marafon DP, Caiello I, et al. High levels of interferon-gamma (IFNγ) in macrophage activation syndrome (MAS) and CXCL9 levels as a biomarker for IFNγ production in MAS. Pediatr Rheumatol. 2015;13(Suppl 1):O84.
  10. Ellis SL, Gysbers V, Manders PM, et al. The cell-specific induction of CXC chemokine ligand 9 mediated by IFN-γ in microglia of the central nervous system is determined by the myeloid transcription factor PU.1. J Immunol. 2010;185(3):1864-1877.
  11. Marucci G, Caiello I, Pardeo M, Messia V, Prencipe G, Pascarella A, De Benedetti F, Bracaglia C. Hemophagocytic Lymphohistiocytosis (HLH) Mimickers: CXCL9 as a potential biomarker distinguishing HLH from other hyperferritinemic syndromes [abstract]. Arthritis Rheumatol. 2018; 70(suppl 10). https://acrabstracts.org/abstract/hemophagocytic-lymphohistiocytosis-hlh-mimickers-cxcl9-as-a-potential-biomarker-distinguishing-hlh-from-other-hyperferritinemic-syndromes/. Accessed May 17, 2019.
  12. Maruoka H, Inoue D, Takiuchi Y, et al. IP-10/CXCL10 and MIG/CXCL9 as novel markers for the diagnosis of lymphoma-associated hemophagocytic syndrome. Ann Hematol. 2014;93(3):393-401. doi:10.1007/s00277-013-1878-y
  13. Metzemaekers M, Vanheule V, Janssens R, Struyf S, Proost P. Overview of the mechanisms that may contribute to the non-redundant activities of interferon-inducible CXC chemokine receptor 3 ligands. Front Immunol. 2018;8:1970. Published 2018 Jan 15. doi:10.3389/fimmu.2017.01970
  14. 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.
  15. Marsh RA, Haddad E. How I treat primary haemophagocytic lymphohistiocytosis. Br J Haematol. 2018;182(2):185-199. doi: 10.1111/bjh.15274.
  1. HOME
  2. About Gamifant
  3. How Gamifant Works
Young girl sitting and grabbing her feet

Gamifant targets a key cytokine In primary HLH1

Gamifant is the only treatment specifically designed to target and neutralize interferon gamma (IFNγ), a key upstream mediator of the proinflammatory cytokine storm associated with primary hemophagocytic lymphohistiocytosis (HLH).1-4

  • Neutralizing IFNγ calms hyperinflammatory symptoms1
  • Gamifant was shown to be effective in patients with refractory, recurrent, or progressive disease or intolerance to conventional therapy1
  • Gamifant is the first and only FDA-approved treatment for primary HLH1,2

Gamifant blocks IFNγ signaLING

Gamifant is a monoclonal antibody that binds to soluble and receptor-bound forms of IFNγ. Binding to IFNγ neutralizes its activity, blocking its intracellular signaling to inhibit macrophage activation and the downstream release of proinflammatory cytokines.1,4-8

Gamifant MOA video See the full video

See Gamifant subdue the storm

Block Ifnγ to subdue the storm

Click through to see how Gamifant blocks IFNγ and subdues the cytokine storm at its center.

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Gamifant bonds to IFNγ to block the activation of macrophages and downstream release of cytokines Gamifant bonds to IFNγ to block the activation of macrophages and downstream release of cytokines

Gamifant was shown to neutralize IFNγ

Although the chemokine CXCL9 is not recommended as a diagnostic marker, nor is it needed to begin treatment or for follow-up monitoring, it is a recognized marker for IFNγ activity. In a clinical trial, sustained and consistent reductions in the plasma concentrations of CXCL9 showed that IFNγ was effectively neutralized by Gamifant.1,6

See the data

CXCL9 is 1 of 3 chemokines in the CXC subfamily predominantly induced by IFNγ. CXCL9 helps regulate immune responses, hematopoietic development, and cell-to-cell communication by acting as a mediator and modulator.10-14

In primary HLH, CXCL9 is produced by IFNγ-activated macrophages. CXCL9 is also a chemoattractant for activated T cells, especially the Th1 cells that produce IFNγ. Thus, higher levels of CXCL9 can indicate IFNγ activity.13

Th1, IFNγ, and CXCL9 all activate each other in a cyclical pattern

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  1. About Gamifant
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Looking for more?

Discover the storm

Primary hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory condition that mostly affects children, but can also occur in adults and teenagers.3,5,14,15

Gamifant reduces the inflammatory symptoms of primary HLH by neutralizing IFNγ.1

Dosing and Administration

Gamifant is given as an intravenous infusion twice per week until the patient no longer requires therapy for the treatment of HLH or until hematopoietic stem cell transplantation (HSCT) is performed.1

ORDERING GAMIFANT

Contact your Sobi Health Systems Director to understand Gamifant ordering options. Connect with your Reimbursement Manager for your individual patient's insurance coverage policy and reimbursement pathway.

Help through the storm

Here you’ll find tools and resources to help your patients and their families.