Emapalumab 10mg/2mL, 50mg/10mL Injection — Interferon-Gamma Inhibitor for Primary Haemophagocytic Lymphohistiocytosis
Emblaveo® (Emapalumab) — First Approved Interferon-Gamma Inhibitor for Primary HLH
Breakthrough Biological Treatment for a Rare and Life-Threatening Immune Disorder
Emblaveo® (Emapalumab) is a fully human monoclonal antibody that neutralises interferon-gamma (IFN-γ) — used to treat primary haemophagocytic lymphohistiocytosis (HLH) in adult and paediatric patients with refractory, recurrent, or progressive disease, or intolerance to conventional HLH therapy. Emblaveo is the first and only approved treatment specifically targeting the IFN-γ pathway — the central driver of the cytokine storm responsible for HLH.
A.K. Pharma is a trusted medicine distributor in Delhi supplying genuine Emblaveo (Emapalumab) to hospitals, haematology centres, paediatric intensive care units, and specialised pharmacies across India. Manufactured by Swedish Orphan Biovitrum (Sobi), Emblaveo represents a significant breakthrough in the management of primary HLH — a disease with very high mortality without prompt and effective treatment.
What is Emblaveo (Emapalumab)?
Emblaveo contains Emapalumab — a fully human IgG1 monoclonal antibody that binds to and neutralises both free and receptor-bound interferon-gamma (IFN-γ). IFN-γ is a pro-inflammatory cytokine that plays a central role in the pathophysiology of primary HLH — driving the uncontrolled immune activation and macrophage hyperactivation that characterise this life-threatening condition.
In primary HLH — caused by genetic defects in cytotoxic T lymphocyte and NK cell function — the inability to kill antigen-presenting cells leads to persistent immune stimulation and massive IFN-γ overproduction. This IFN-γ excess drives macrophage activation, haemophagocytosis (engulfment of blood cells by macrophages), and a systemic inflammatory storm causing multi-organ failure and death if untreated.
By neutralising IFN-γ, Emblaveo directly addresses the central pathological mechanism of primary HLH — reducing immune activation, suppressing haemophagocytosis, and controlling the cytokine storm to allow patients to reach haematopoietic stem cell transplantation (HSCT) — the only curative treatment.
Full prescribing information is available at the FDA label for Emapalumab.
Understanding Primary HLH — A Life-Threatening Immune Emergency
Primary HLH (also called familial HLH or genetic HLH) is a rare, autosomal recessive disorder caused by mutations in genes encoding proteins essential for cytotoxic lymphocyte function including:
These genetic defects impair cytotoxic killing of antigen-presenting cells — leading to:
Without treatment primary HLH is rapidly fatal — median survival without therapy is less than 2 months. Even with conventional HLH-94/HLH-2004 therapy (dexamethasone + etoposide ± cyclosporine) a significant proportion of patients are refractory or intolerant, creating a critical unmet need that Emblaveo addresses.
For detailed HLH information refer to the Histiocyte Society HLH guidelines.
Clinical Studies and Evidence
Pivotal Trial NI-0501-04 (Emapalumab in Refractory Primary HLH) Published in the New England Journal of Medicine (2020), this pivotal single-arm trial demonstrated that Emapalumab achieved an overall response rate of 63% in paediatric patients with refractory, recurrent, or progressive primary HLH who had failed conventional HLH therapy. Critically 65% of patients were able to proceed to haematopoietic stem cell transplantation — the curative treatment for primary HLH — demonstrating Emblaveo’s ability to bridge patients to transplant.
Key Trial Findings:
Long-Term Follow-Up Data Long-term follow-up data from the pivotal trial demonstrated durable responses with Emapalumab and confirmed the safety profile across extended treatment periods — supporting use as a bridge to HSCT.
Combination with Dexamethasone All patients in the pivotal trial received Emapalumab in combination with dexamethasone — with the combination demonstrating synergistic immunosuppressive activity superior to either agent alone in controlling HLH.
Available Strengths
Emblaveo is available as:
Treatment is initiated at 1mg/kg twice weekly by IV infusion and may be increased based on clinical response and IFN-γ levels. Emblaveo is always used in combination with dexamethasone.
Indications — What Emblaveo is Used For
Note: Emblaveo is indicated specifically for PRIMARY (genetic) HLH — not secondary HLH (associated with malignancy, infection, or autoimmune disease). Genetic confirmation of primary HLH should be sought before initiating treatment.
For detailed indication information refer to NCI Emapalumab Drug Information and Orphanet HLH information.
Key Benefits of Emblaveo
First Approved IFN-γ Inhibitor Emblaveo is the first and only approved medicine specifically targeting IFN-γ — the central pathological driver of primary HLH — providing a mechanistically targeted approach superior to broad immunosuppression.
Bridges Patients to Curative HSCT The primary goal of HLH treatment is to control disease sufficiently to allow haematopoietic stem cell transplantation — the only cure. Emblaveo achieves this bridge to HSCT in 65% of previously refractory patients — a critical clinical achievement in this life-threatening disease.
Rapid IFN-γ Neutralisation IFN-γ levels are reduced by more than 95% within days of the first Emblaveo infusion — providing rapid control of the cytokine storm driving HLH inflammation.
Effective in Patients Failing Conventional Therapy Emblaveo specifically addresses the unmet need in patients who are refractory to or intolerant of conventional HLH-94/2004 therapy (dexamethasone + etoposide) — providing a treatment option where previously none existed.
Validated HLH Biomarker Monitoring IFN-γ levels serve as a validated pharmacodynamic biomarker for Emblaveo — enabling objective monitoring of treatment response and dose adjustment decisions.
How Emblaveo Works
In primary HLH the central pathological mechanism involves:
Emblaveo interrupts this cycle by:
The result is disease control sufficient to bridge patients to the only curative treatment — haematopoietic stem cell transplantation.
For a detailed mechanism overview refer to the Histiocyte Society and European Haematology Association HLH guidelines.
HLH Diagnostic Criteria — HScore and HLH-2004
Primary HLH is diagnosed when 5 or more of the following 8 criteria are met (HLH-2004 criteria):
Genetic testing confirms primary (familial) HLH — essential for Emblaveo indication.
Dosage and Administration
Starting Dose:
Dose Escalation Based on Response:
Monitoring:
Duration:
Administration:
Full dosing guidelines available at Drugs.com Emapalumab Dosage.
Who Should Use Emblaveo
Emblaveo is prescribed for:
Emblaveo is prescribed by haematologists, paediatric haematologists, and immunologists at specialised centres. A.K. Pharma supplies Emblaveo to hospitals, haematology centres, and paediatric ICUs across Delhi and India.
Possible Side Effects
Common side effects include infections (particularly histoplasmosis and other opportunistic infections), pyrexia, hypertension, and infusion-related reactions.
Serious side effects include:
Full side effect information available at FDA Emapalumab Safety Information.
Precautions
Storage and Handling
As a responsible medicine distributor in Delhi, A.K. Pharma maintains strict cold chain requirements during storage and supply of Emblaveo ensuring product integrity for every unit supplied.
Manufacturer Information
Emblaveo (Emapalumab) is manufactured by Swedish Orphan Biovitrum AB (Sobi), a speciality biopharmaceutical company focused on rare diseases. Emapalumab received FDA approval in November 2018 as the first treatment approved for primary HLH. A.K. Pharma supplies only genuine Emblaveo sourced from authorized Sobi distributors.
Related Immunosuppressant and Haematology Medicines Available at A.K. Pharma
Frequently Asked Questions
Q. What is Emblaveo used for? Emblaveo (Emapalumab) is used to treat primary haemophagocytic lymphohistiocytosis (HLH) — a rare and life-threatening immune disorder — in patients with refractory, recurrent, or progressive disease or intolerance to conventional therapy. More information available at the Histiocyte Society.
Q. What is the generic name of Emblaveo? The generic name of Emblaveo is Emapalumab. It is a fully human monoclonal antibody that neutralises interferon-gamma (IFN-γ).
Q. What is HLH? HLH (Haemophagocytic Lymphohistiocytosis) is a rare, life-threatening condition caused by uncontrolled immune activation and cytokine storm. Primary HLH is caused by genetic defects in cytotoxic lymphocyte function. Without treatment it is rapidly fatal — median survival less than 2 months without therapy.
Q. How does Emblaveo differ from conventional HLH treatment? Conventional HLH treatment (dexamethasone + etoposide per HLH-94/2004 protocol) provides broad immunosuppression. Emblaveo specifically targets IFN-γ — the central pathological driver of HLH — providing a mechanistically targeted approach that is effective even in patients who have failed conventional therapy.
Q. Is Emblaveo a cure for HLH? Emblaveo is not a cure for primary HLH — haematopoietic stem cell transplantation (HSCT) is the only curative treatment. Emblaveo controls HLH disease activity sufficiently to allow patients to proceed to HSCT — the critical bridge-to-transplant role that makes it life-saving.
Q. Is Emblaveo available in India? Emblaveo can be supplied to hospitals, haematology centres, and paediatric ICUs across India through licensed pharmaceutical distributors. Contact A.K. Pharma — medicine distributor in Delhi — for availability and pricing.
Q. What is the price of Emblaveo in India? Emblaveo price in India varies by vial size and treatment duration. Contact A.K. Pharma at 011 4172 6999 or WhatsApp +91 9810034827 for current pricing and supply information.
Q. How to order Emblaveo from A.K. Pharma? You can request a quote directly from this page, call us at 011 4172 6999, or WhatsApp us at +91 9810034827 with your requirements and we will respond promptly.
Q. Does A.K. Pharma supply Emblaveo in bulk? Yes. A.K. Pharma supplies Emblaveo to hospitals, haematology centres, and paediatric ICUs across Delhi and India. Contact us for pricing and availability.
Why Order Emblaveo from A.K. Pharma?
Contact A.K. Pharma for Emblaveo Supply 📍 E-2/257A, 2nd Floor, Shastri Nagar, New Delhi 110052 📞 011 4172 6999 📱 WhatsApp: +91 9810034827 🌐 akpharma.in
