Miscellaneous immune disorders:
Indications for GAMIFANT:
Primary hemophagocytic lymphohistiocytosis (HLH) in adults and children (newborn and older) with refractory, recurrent or progressive disease or intolerance with conventional HLH therapy.
Adults and Children:
Give prophylaxis for herpes zoster, Pneumocystis jirovecii, and fungal infections prior to infusion. Also, begin dexamethasone ≥5–10mg/m2 daily the day before starting Gamifant; dose can be tapered as appropriate. Give by IV infusion over 1hr. Initially 1mg/kg twice weekly (every 3–4 days) until HSCT performed or unacceptable toxicity. Subsequent doses may be increased based on response. Dose modification: see full labeling.
Monitor closely for serious infections (eg, sepsis, pneumonia, bacteremia); and those caused by specific pathogens favored by IFN-gamma neutralization (including mycobacteria, herpes zoster, histoplasma capsulatum). Evaluate for TB risk factors and test for latent TB prior to treatment start. Give TB prophylaxis if patient at risk, has (+) PPD test result or (+) IFN-gamma release assay. Monitor for TB, adenovirus, EBV, and CMV every 2 weeks and as clinically indicated. Monitor for infusion-related reactions. Pregnancy. Nursing mothers.
Interferon gamma blocking antibody.
Avoid concomitant live or live attenuated vaccines for at least 4 weeks after the last dose. May reduce efficacy of CYP450 substrates: monitor and adjust dose of these drugs.
Infections, hypertension, infusion-related reactions, pyrexia, hypokalemia, constipation, rash, abdominal pain, diarrhea, lymphocytosis, cough, irritability, tachycardia, tachypnea.