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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:10.1182/blood-2011-03-278127
  2. Marsh RA, Haddad E. How I treat primary haemophagocytic lymphohistiocytosis. Br J Haematol. 2018;182(2):185-199. doi: 10.1111/bjh.15274
  3. Price B, Lines J, Lewis D, Holland N. Haemophagocytic lymphohistiocytosis: a fulminant syndrome associated 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
  4. Gamifant (emapalumab-lszg) prescribing information. Stockholm, Sweden: Sobi, Inc. 2022.
  5. 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
  6. HLH diagnosis strategy. Cincinnati Children’s Hospital. March 29, 2024. https://www.cincinnatichildrens.org/service/h/hlh/clinical/test
  7. Henter J, Horne A, Aricò M, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48(2):124–131. doi:10.1002/pbc.21039
  8. Jordan MB, Allen CE, Greenberg J, et al. Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: recommendations from the North American Consortium for Histiocytosis (NACHO). Pediatr Blood Cancer. 2019;66(11):e27929. doi:10.1002/pbc.27929
  9. Morimoto A, Nakazawa Y, Ishii E. Hemophagocytic lymphohistiocytosis: pathogenesis, diagnosis, and management. Pediatr Int. 2016;58(9):817-825. doi:10.1111/ped.13064
  10. La Rosée P. Alleviating the storm: ruxolitinib in HLH. Blood. 2016;127(13):1626-1627. doi:10.1182/blood-2016-02-697151
  1. HOME
  2. Primary HLH
  3. About Primary HLH

There are 3 recognized options for identifying this rare condition2,4-6:

Checklist clipboard icon Fulfillment of 5 of the 8 HLH-2004 criteria in the absence of an underlying cause, such as malignancy, especially lymphoma, or viral infection
DNA icon A positive genetic test for mutations associated with primary HLH
Family icon Family history consistent with primary HLH
  • Fever
  • Hypertriglyceridemia (fasting, ≥265 mg/dL) and/or hypofibrinogenemia (≤1.5 g/L)
  • Splenomegaly
  • Hemophagocytosis* in bone marrow, spleen, or lymph nodes
  • Cytopenias (affecting at least 2 of 3 lineages in the peripheral blood)
    • Hemoglobin <90 g/L
    • Platelets <100 x 109/L (in infants <4 weeks: hemoglobin <100 g/L)
    • Neutrophils <1.0 x 109/L
  • *Note that hemophagocytosis is not specific nor always present in early stages of the disease.
  • Fever
  • Hypertriglyceridemia (fasting, ≥265 mg/dL) and/or hypofibrinogenemia (≤1.5 g/L)
  • Splenomegaly
  • Hemophagocytosis* in bone marrow, spleen, or lymph nodes
  • Cytopenias (affecting at least 2 of 3 lineages in the peripheral blood)
    • Hemoglobin <90 g/L
    • Platelets <100 x 109/L (in infants <4 weeks: hemoglobin <100 g/L)
    • Neutrophils <1.0 x 109/L
  • Ferritin ≥500 μg/L
  • Low or absent natural killer (NK)-cell activity
  • Soluble CD25 (interleukin [IL]-2 receptor) >2400 U/mL (or per local reference laboratory)
*Note that hemophagocytosis is not specific nor always present in early stages of the disease.8
  • FHL3 - UNC13D
  • X-linked lymphoproliferative disorder 1
  • FHL2 - PRF1
  • X-linked lymphoproliferative disorder 2
  • FHL1 - Unknown
  • Griscelli syndrome type 2 (RAB27A)
  • FHL5 - STXBP2 (UNC18B)
  • Chediak-Higashi syndrome - LYST
  • FHL4 - STX11

Keep in mind that not all genetic causes that can lead to HLH have been identified. This is an area that is continually being studied.

Accelerate diagnosis with alternatives to genetic testing6

When it comes to treating primary HLH, there is no time to wait. Prior to receiving the results of a genetic test, ancillary testing and flow cytometry can be used, where available, to help differentiate between primary and secondary HLH.6

Learn more

IFNγ is a driver of primary HLH9,10

IFNγ is central to the "cytokine storm"—an uncontrolled release of inflammatory cytokines and overactivation of phagocytes that give the syndrome its name.9,10

Learn more

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  2. About Primary HLH

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  2. Testing for Primary HLH

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.1,5

Gamifant controls the inflammatory symptoms of primary HLH by neutralizing IFNγ.4

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.4

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.