about Primary HLH
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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.
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.
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Primary hemophagocytic lymphohistiocytosis (HLH) is a rare genetic disease characterized by life-threatening inflammatory symptoms.1,2 Though primary HLH can affect adults and teenagers, the majority of patients are very young children—often in the first months or years of their lives.1,3 Due to the rapidly progressive nature of the disease, immediate therapy is required to temper the “cytokine storm”—a massive release of cytokines that leads to the signs and symptoms of primary HLH.1,4
Primary HLH presents as a heterogeneous syndrome of rapidly progressive, life-threatening inflammatory symptoms, including1,2:
To date, general consensus in the scientific community on primary HLH is as follows1,3:
PRIMARY HLH | |
---|---|
Mortality rate | High mortality rate |
Clinical presentation | Heterogeneous |
Age of presentation | Typically infants/early childhood; documented cases also exist in teens and adults |
Cause | Testable genetic mutation |
Family history | May be positive for the disease |
Recurrence | Likely |
Resolution | Usually requires hematopoietic stem cell transplantation (HSCT) |