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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.
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.
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.
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.
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, 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.
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.
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