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Gamifant (emapalumab-lzsg) is an interferon gamma (IFNγ)-neutralizing antibody indicated for the treatment of adult and pediatric (newborn and older) patients with:
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.
Gamifant (emapalumab-lzsg) is an interferon gamma (IFNγ)-neutralizing antibody indicated for the treatment of adult and pediatric (newborn and older) patients with:
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.
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 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.
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%).
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%).
When it comes to treating primary hemophagocytic lymphohistiocytosis (HLH), there’s no time to wait. Prior to receiving genetic testing results, other assessments can help you determine the likelihood of HLH so that you can initiate treatment as soon as possible.1,2
Decreased levels of the
following can help identify a genetic cause3
CD=cluster of differentiation; SLAM=signaling lymphocytic activation molecule; XIAP=X-linked inhibitor of apoptosis protein.
Flow cytometry for perforin expression and CD107a have high specificity for identifying primary
HLH3
Quantitative viral PCRs:
EBV, CMV, adenovirus, HSV 1, HSV 2, etc
CT of chest, abdomen, and neck
Testing for tick- or mosquito-borne diseases
PET-CT
followed by lymph node biopsy to evaluate for lymphoma
MRI of brain
Lyme disease
CMV=cytomegalovirus; CT=computed tomography; EBV=Epstein-Barr virus; HSV=herpes simplex
virus;
MRI=magnetic resonance imaging; PCR=polymerase chain reaction; PET=positron emission tomography.
These tests can help eliminate other conditions and/or define treatable underlying
triggers for
HLH2-5
Chemokine (C-X-C motif) ligand 9 (CXCL9) is a type of cytokine released almost exclusively by interferon gamma (IFNγ)-activated macrophages. The primary function of CXCL9 is to attract T cells into inflamed tissues.6
CXCL9 production is induced predominantly by IFNγ. Evidence suggests CXCL9 is a potential biomarker for IFNγ activity.1
There is growing recognition of testing for CXCL9 as a biomarker for IFNγ activity.1,6 The following organizations offer CXCL9 testing to help with identifying primary HLH:
| Machaon Diagnostics | |
|---|---|
| Website | www.machaondiagnostics.com/test/cxcl9-level |
| Turnaround time | STAT: <24 hours Routine: <1 week |
| Lab hours | 24/7 |
| Phone | 1-800-566-3462 510-839-5600 |
| Fax | 510-839-6153 |
| Cincinnati Children’s Hospital | |
|---|---|
| Website | www.testmenu.com/cincinnatichildrens/Tests/723501 |
| Turnaround time | 4 days |
| Lab hours | Mon-Fri, 8:00 AM to 5:00 PM (ET) |
| Phone | 513-636-4685 |
| Fax | 513-636-3861 |
This is not an exhaustive list of labs offering CXCL9 testing. Please check for the availability of this test within your own institution prior to contacting these sites.
ALT=alanine transaminase; IL-18=interleukin-18; sCD25=soluble CD25.
In the absence of an underlying cause, such as malignancy, these tests can be useful for
identifying the typical features of primary HLH.3
Gamifant was shown to neutralize IFNγ as measured by reductions in the plasma concentration of CXCL9.7