Nivestym Drug Information: How It Works, Who Needs It, and What To Expect

Published on by Dr. Mark Alfonso, PharmD, BCMTMS

In the U.S., about 2 million new cancer cases are diagnosed each year. Chemotherapy is the most common and effective treatment for cancer, but it comes with many side effects. One such side effect is that it causes a condition known as neutropenia, in which the level of neutrophils (a type of white blood cell) becomes very low. When this happens, you become more likely to develop infections, which can be fatal if not managed well. 

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Fortunately, drugs such as Nivestym (filgrastim-aafi) can help restore white blood cells after chemotherapy. In this article, we’ll talk about everything you need to know about Nivestym in a clear and easy-to-understand way.

What Is Nivestym?

Nivestym (filgrastim-aafi) is an FDA-approved medicine used to treat neutropenia (low levels of neutrophils, a type of white blood cell) caused by chemotherapy, bone marrow transplant, or some other condition. It is a synthetic version of the naturally occurring protein called granulocyte colony-stimulating factor (G-CSF), which promotes white blood cell production. 

The FDA first approved Nivestym on July 20, 2018, as a biosimilar to Neupogen [1]. This means that there is no clinical difference between Nivestym and Neupogen, but Nivestym is less expensive.  

What Is Nivestym Used To Treat?

Nivestym (filgrastim-aafi) is a medicine that helps your body make more white blood cells, which help fight infections. It is FDA-approved for the following purposes [2][3]:

Chemotherapy-Induced Neutropenia: This medication can reduce the risk of infection in cancer patients receiving intensive chemotherapy that can lower white blood cell counts and cause fever.

Acute Myeloid Leukemia: Nivestym can help white blood cells recover faster and shorten the duration of fever in patients with acute myeloid leukemia (AML) after extensive chemotherapy.

Bone Marrow Transplant: After a bone marrow transplant, Nivestym can speed up white blood cell recovery and reduce the duration of severe neutropenia. 

Peripheral Blood Progenitor Cell Collection: Nivestym is used to move blood-forming stem cells into the bloodstream so they can be collected and used later for treatment.

Severe Chronic Neutropenia: Nivestym can help patients with long-term neutropenia, including congenital neutropenia‚ cyclic neutropenia‚ or idiopathic neutropenia. It can reduce infections and related problems, such as fever and mouth sores.

How Does Nivestym Work?

Neutrophils are a kind of white blood cell that fight bacterial and fungal infections. They are produced in your bone marrow. Unfortunately, chemotherapy and radiation therapy can harm your bone marrow, which can reduce or stop neutrophil production. This increases your chance of having serious infections.

Nivestym is a synthetic version of a naturally occurring protein called granulocyte colony-stimulating factor (G-CSF). It works by encouraging your bone marrow to:

  • Produce more neutrophils
  • Speed up neutrophil production
  • Release neutrophils into the bloodstream

As a result, your neutrophil count increases faster, reducing the time your immune system is weakened after chemotherapy. 

Side Effects

Like any other medication, Nivestym can also cause some side effects. Most are mild to moderate, but some can be severe. Here are some of the side effects of Nivestym [2][3]:

Common Side Effects

Patient experiencing muscle pain, a side effect of using Nivestym
  • Bone Pain
  • Muscle Pain
  • Headache
  • Fatigue
  • Nausea
  • Injection site reaction (redness, swelling, or pain)
  • Diarrhea and vomiting

Bone pain is the most common side effect because your bone marrow is working harder to produce more white blood cells. It is usually manageable with mild pain medicines. 

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Severe Side Effects

Serious side effects from Nivestym are rare but can happen. Here are some serious side effects of Nivestym:

Spleen Rupture: In rare cases, Nivestym can cause rupture in your spleen, which can be fatal. Symptoms include pain in the upper left abdomen or shoulder.

Acute Respiratory Distress Syndrome (ARDS): Nivestym can cause ARDS in some patients. Symptoms include shortness of breath, rapid breathing, and low oxygen levels.

Serious Allergic Reactions: You may experience allergic reactions to Nivestym, particularly with the initial dose. Symptoms include rash, swelling of the face or throat, dizziness, or difficulty breathing. 

Capillary Leak Syndrome: This is a rare but serious condition where fluid leaks from your blood vessels. This leads to low blood pressure and swelling.

Sickle Cell Disorders: Nivestym can cause sickle cell crisis in patients with sickle cell disorder. This can be fatal. Discontinue Nivestym if a sickle cell crisis occurs.

Low Platelet Count: Nivestym can lower your platelet count, which can be severe.

Kidney Problems: Nivestym can cause inflammation in your kidneys. Symptoms include dark-colored urine and swelling in the face, hands, or feet.

Please note that this is not a complete list of all possible side effects. Consult your doctor immediately if you experience any severe side effects of Nivestym.

Dosing Information

The dosage of Nivestym depends on your body weight and medical condition. Your doctor will determine the right dosage for you based on your condition. Here is the usual recommended dosage [3]:

For Cancer Patients Receiving Chemotherapy (including AML treatment): The usual starting dose is 5 micrograms per kilogram of your body weight per day (5 mcg/kg/day). 

For Cancer Patients Undergoing a Bone Marrow Transplant: The recommended dose is 10 mcg/kg/day. It is given as an IV infusion that lasts no longer than 24 hours.

For Patients Undergoing Autologous Stem Cell Therapy: The recommended dose is 10 mcg/kg/day. You will receive it as an injection under the skin. Your doctor will start at least 4 days before the first stem cell collection and continue until the final collection is completed.

For Patients With Congenital Neutropenia: The starting dose is 6 mcg/kg. You will receive it via an injection under your skin twice daily.

Patients with Cyclic or Idiopathic Neutropenia: The recommended starting dose is 5 mcg/kg/day.

Before Starting Nivestym

Before you start your treatment with Nivestym, have a detailed discussion with your doctor to ensure that the medicine is safe and appropriate. Tell your doctor if you:

  • Have osteoporosis (a bone disease that causes bones to become weak and prone to fracture)
  • Have allergies to filgrastim or pegfilgrastim
  • Have sickle cell disease because Nivestym can trigger life-threatening complications.
  • Have a history of bone marrow disorders
  • Have kidney problems
  • Have ever had an enlarged spleen or splenic rupture
  • Experience difficulty breathing or cough
  • Are pregnant or planning to become pregnant. There is limited data on its safety during pregnancy. You should only use this medication during pregnancy if your doctor thinks the potential benefits outweigh the risks.
  • Are breastfeeding
  • Are taking other prescription medicines, over-the-counter drugs, or herbal supplements

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Cost

Nivestym is available in single-dose vials and prefilled syringes in various strengths. One common option is 300 mcg/ml, where a package of 10 single-dose vials (1 ml each) costs around $2,147 [4]. However, the actual price depends on your insurance plan, location, and the pharmacy you visit. Contact us if you are interested in exploring financial assistance options for Nivestym.

IVIG for Hematologic Malignancy-Associated Secondary Immunosuppression: Key Insights, Based on the Latest Evidence

Published on by Dr. Robert Hakim, PharmD

IVIG for hematologic malignancy-associated secondary immunosuppression may be an option when patients have severe or recurrent infections. Discover how IVIG is administered and find answers to the most common questions. 

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Highlights

  1. Hematologic malignancy-associated secondary immunosuppression means a weakened immune system caused by hematologic malignancies or their treatments. 
  2. IVIG is widely used to prevent recurrent and severe infections in patients with hematologic malignancies. 
  3. A patient may switch from IVIG to SCIG in certain situations. 

What Is Hematologic Malignancy-Associated Secondary Immunosuppression?

Let’s first break down the term “hematologic malignancy-associated secondary immunosuppression.” 

Hematologic Malignancy

These cancers originate in the bone marrow or the cells of the immune system. The three main groups of hematologic malignancy are:

  1. Leukemia: Leukemia occurs when the bone marrow produces abnormally high amounts of white blood cells. 
  2. Lymphoma: Lymphoma originates in the part of the immune system called the lymphatic system. Lymphoma affects white blood cells known as lymphocytes. 
  3. Multiple myeloma: This is a rare cancer that causes an overgrowth of specialized white blood cells called plasma cells. 

Secondary Immunosuppression

Secondary immunosuppression refers to a weakened immune system due to:

  • Disorders of blood or bone marrow
  • Medications
  • Cancer treatments
  • Certain cancers
  • Malnutrition 
  • Infectious diseases

Hematologic malignancy-associated secondary immunosuppression occurs when hematologic malignancies or their treatments cause a weakened immune system. A weakened immune system puts patients at a higher risk of getting recurrent or severe infections. 

IVIG (intravenous immunoglobulin) is one of the treatment options for hematologic malignancy-associated immunosuppression. Other management options include:

  • Preventive antibiotic treatment and vaccination
  • Reduced immunosuppression
  • Treatment of the underlying condition

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IVIG for Hematologic Malignancy-Associated Secondary Immunosuppression: What We Know So Far

Smiling IVIG nurse standing next to an IV pole

IVIG is widely used to help reduce the risk of severe infections in patients with hematologic malignancies. In these patients, IVIG can prevent recurrent and severe infections [1]. A healthcare provider may consider IVIG for hematologic malignancy-associated secondary immunosuppression when [2,3]:

  • Patients have severe or recurrent infections
  • Antibiotics are ineffective
  • IgG level is less than 4g/l
  • Patient is receiving CAR-T Cell therapy

Further Reading: IVIG for CAR-T Therapy-Related Hypogammaglobulinemia: Uses, Benefits, Dosage, and More

However, the use of IVIG for hematologic malignancy-associated secondary immunosuppression is still not clear because:

  • The benefits of IVIG for hematologic malignancy-associated secondary immunosuppression come from small clinical trials; robust evidence from large controlled trials is lacking. 
  • There are no specific clinical treatment guidelines on the use of IVIG in this patient population.

Due to cost and limited supply of IVIG, it is typically recommended for therapy to be individualized based on the patient’s condition and type of cancer therapy. 

Treatment Considerations and Dosing

Administering IVIG for hematologic malignancy-associated secondary immunosuppression involves several steps. 

First, a healthcare provider will measure the IgG levels before or during cancer treatment. Second, they will determine when to start IVIG therapy, which typically relies on the IgG levels. Lastly, they will determine the dose based on the body weight. 

Step 1: Measuring IgG Levels

According to the European expert consensus, in patients with hematologic malignancies [4]:

  • Measuring IgG levels before starting cancer treatment can help estimate the patient’s risk of developing infections. 
  • A healthcare provider may also measure Ig levels during routine visits if the patient has already started cancer treatment. 

For patients younger than 21 years, a healthcare provider will interpret the IgG levels based on what’s considered normal for their specific age. 

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Step 2: Starting IVIG Therapy

The ideal candidates for IVIG for hematologic malignancy-associated secondary immunosuppression are those with:

  • IgG levels less than 4 g/l who have received antimicrobial therapy. IVIG should be started during or after a single severe infection or recurrent infection. 
  • IgG levels less than 4 g/l who have severe or recurrent infections even after they have received antimicrobial therapy. 

While not all experts agree, patients with IgG levels between 4 g/l and 6 g/l who have severe or recurrent infections even after they have received antimicrobial therapy may also receive IVIG treatment. 

Step 3: IVIG Dosing

IVIG dose depends on body weight. If the patient is obese, dosage adjustment according to the ideal body weight specific to the patient is necessary.

The dosing regimen of IVIG for hematologic malignancy-associated secondary immunosuppression is as follows [3]:

  • The starting dose of IVIG is 0.4 g/kg body weight every 3 to 4 weeks. 
  • The maintenance dose of IVIG is 0.4 to 0.8 g/kg body weight every 3 to 4 weeks. 

IVIG for Hematologic Malignancy-Associated Secondary Immunosuppression: Special Considerations

  • When starting IVIG therapy, a healthcare provider may consider stopping antimicrobial treatment if the infection is under control. 
  • If the patient doesn’t have an infection for a significant period of time (typically 6 months), a healthcare provider may stop IVIG therapy. 
  • Patients who no longer use IVIG should get their IgG levels tested during routine visits. 
  • Should severe or persistent infections recur in patients who no longer use IVIG, IVIG treatment can be restarted. 
  • IVIG typically causes mild infusion-related reactions, such as headache, nausea, and muscle aches. 
  • Rare but severe side effects include severe allergic reactions, kidney failure, and rupture of red blood cells. A healthcare provider will closely monitor patients during IVIG therapy if they have risk factors for these serious side effects.

IVIG for Hematologic Malignancy-Associated Secondary Immunosuppression: Can A Patient Switch to Subcutaneous Immune Globulin (SCIG)? 

A patient can switch to SCIG after discussing the potential benefits and risks with their healthcare provider. The major benefit is that they can self-administer SCIG at home after proper training. SCIG typically also has fewer side effects than IVIG. They may choose to switch to SCIG if:

  • They are ready to take charge of their health.
  • Other forms of treatment fail to provide adequate IgG levels.
  • Specific immunoglobulin formulations are unavailable.

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Frequently Asked Questions

What hematologic malignancy is associated with secondary immunosuppression?

Chronic lymphocytic leukemia and multiple myeloma are often associated with secondary immunosuppression. 

Which type of virus has been associated with hematologic malignancies?

Viruses associated with hematologic malignancies include HIV, Epstein–Barr virus (EBV), and Kaposi sarcoma–associated herpesvirus (KSHV), also known as human herpesvirus 8 (HHV-8).

What is the most common cause of secondary immunodeficiency?

The most common causes of secondary immunodeficiency are:

  • Blood or bone marrow disorders
  • Cancer treatments
  • Certain cancers

Altuviiio: A Once-Weekly Treatment for Hemophilia A

Published on by Dr. Christine Leduc, PharmD

FDA-approved in February 2023, Sanofi’s Altuviiio is a long-acting antihemophilic factor (recombinant) used to treat hemophilia A in children and adults. Learn about its mechanism of action, dosage, side effects, cost, and more. 

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Important Warnings and Precautions

Allergic Reactions

This medication may cause allergic reactions, which can be fatal. Seek emergency medical care if you experience:

  • Shortness of breath
  • Wheezing
  • Hives
  • Chest tightness
  • Low blood pressure
  • Itching

Inhibitor Production

In some cases, your body can make proteins called “inhibitors”, which can stop this medication from working as intended. 

If your immune system makes inhibitors, you will need special treatment to help control bleeding episodes. Talk to your healthcare provider to learn more about inhibitors and tests to measure your inhibitor level. 

Before Taking Altuviiio

Before you receive your first dose, inform your provider if you:

  • Have or had any medical issues.
  • Are allergic to this product, any product component, or any other medications.
  • Are pregnant or planning to get pregnant. It is unknown if this medication harms your unborn baby. 
  • Are breastfeeding. It is unknown if this medication is present in breast milk, and if it can harm the baby. 

Altuviiio Introduction and Uses

Altuviiio injection is a brand-name prescription product. It contains the active ingredient “recombinant clotting factor VIII”, also called the recombinant antihemophilic factor. Altuviiio belongs to a class of medications called antihemophilic factors. 

A healthcare provider may prescribe this medication for children and adults with hemophilia A to: 

  • Control and prevent bleeding episodes
  • Prevent bleeding during surgery
  • Reduce the frequency of bleeding episodes

In people with hemophilia A, blood doesn’t clot properly because they have a deficiency of coagulation factor VIII. Factor VIII deficiency increases the risk of unexplained bleeding or, in severe cases, potentially fatal internal bleeding. 

This medication shouldn’t be used to treat another similar bleeding disorder called von Willebrand disease.

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Altuviiio Mechanism of Action

Blood sample tube and paper order request for factor VIII test analysis

Three components in Altuviiio work together to stop the bleeding. They are:

  • Fc Fusion, which helps Factor VIII recirculate in the blood. 
  • XTEN Technology, which helps prevent the early breakdown of Factor VIII. 
  • vWF Fragments, which help Factor VIII stay longer in the blood.

Altuviiio’s half-life is three to four times greater than other Factor VIII products. This means the medication remains in the body for longer, enabling a once-weekly dosing schedule. 

Altuviio Dosage

This medication is supplied as a sterile white-to-off-white powder for reconstitution prior to intravenous (IV) injection in single-dose vials. 

The following strengths are available in the US: 

  • 250 IU
  • 500 IU
  • 750 IU
  • 1,000 IU
  • 2,000 IU
  • 3,000 IU
  • 4,000 IU

Before administering this medication, your healthcare provider will use a specific formula to determine the dose. Then, they will prepare the liquid for injection according to the instructions on the package. 

The dose and duration of treatment depend on:

  • Body weight
  • Severity of factor VIII deficiency
  • Desired factor VIII level

Dosing for Routine Prophylaxis (Prevention)

For children and adults: 50 IU/kg of Altuviiio administered once weekly. 

Dosing for Control of Bleeding Episodes

  • Minor and moderate bleeding: Single dose of 50 IU/kg. 
  • Major bleeding: Single dose of 50 IU/kg. 

Dosing for Preventing Bleeding During Surgery

  • Minor surgery: Single dose of 50 IU/kg. An additional dose of 30 or 50 IU/kg may be considered 2 to 3 days after surgery (post-operative dose).
  • Major surgery: Single dose of 50 IU/kg. If needed, a healthcare provider may administer additional doses of 30 or 50 IU/kg every 2 to 3 days.

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Altuviiio Side Effects

Headache and joint pain are the most common side effects. Inform your healthcare provider if other side effects occur. 

Altuviiio Proper Use

Your healthcare provider will teach you or your caregiver how to mix and administer Altuviiio when used for the first time. 

Important Things To Know Before Injecting Altuviiio

  • Read all instructions carefully. If you have any questions, talk to your healthcare provider. 
  • Before injecting, you should mix Altuviiio powder with liquid that comes in a prefilled syringe. 
  • The solution is administered intravenously. 
  • Don’t use Altuviiio if it has been damaged, the solution is cloudy, or if the syringe cap is not securely attached. 

Altuviiio Storage and Handling 

Before Preparing the Solution

  • Store in the original package.
  • Maintain storage temperature between 2°C and 8°C (36°F and 46°F). Avoid freezing.
  • You may store this medication at room temperature (maximum temperature 30°C or 86°F) for up to 6 months in a row.
  • After you remove the product from the refrigerator, allow the vial and prefilled syringe to come to room temperature before injecting. 
  • Never put the vial or prefilled syringe in hot water. 
  • Once at room temperature, avoid returning the product to the refrigerator.
  • Keep the product away from direct sunlight. 

After Preparing the Solution

  • Avoid direct sun exposure. 
  • Use the solution within 3 hours after mixing. 
  • Don’t refrigerate after preparing the solution. 

Altuviiio Cost

Cost can vary depending on your insurance plan, location, and pharmacy. Contact your insurance provider to determine whether your plan covers this medication and whether prior authorization is required.

Sanofi Corporation, the manufacturer of Altuviiio, offers financial assistance through its HemAssist™ program. You can also enroll in the copay program and the free-trial program. 

Click here to talk about your options with one of our financial assistance specialists. 

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Altuviiio vs. Hemlibra

Both medicines are used to help prevent bleeding in people with hemophilia A. But these medicines differ in their mechanism of action and route of administration. According to a 2024 study, Altuviiio may be superior to Hemlibra in preventing bleeding in certain patients with hemophilia A [1]. Your healthcare provider will help determine the most appropriate medication for your condition.

IVIG For Major Surgery-Associated Secondary Immunosuppression

Published on by Dr. Robert Hakim, PharmD

After major surgical procedures like organ transplantation or open-heart surgery, your body goes through a lot more than what you see on the outside. The physical stress of the procedures can weaken your immune system, which is also known as major surgery-associated secondary immunosuppression or surgery-induced secondary immunosuppression. 

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Secondary immunosuppression after major surgery also raises the risks of serious complications like pneumonia, bloodstream infection (sepsis), or delayed wound healing in many patients. Intravenous immunoglobulin (IVIG) is sometimes used as an adjunct therapy to support the immune system during this vulnerable period. 

Read on to learn what surgery-related immunosuppression is, what recent studies show, how IVIG may help, and who may benefit from this treatment.

Major Surgery-Associated Secondary Immunosuppression: Overview

Major surgery-associated secondary immunosuppression occurs as a consequence of an external factor (such as surgery and the physical stress it creates), not by a disease or primary immune defect that the patient may already have. 

Generally, when a person undergoes major surgery such as heart surgery, organ surgery, trauma repair, or a major abdominal operation, their body experiences drastic immunological changes. 

The immune system reacts in two stages: the hyperinflammation phase and the compensatory anti-inflammatory phase (CARS). 

In the hyperinflammation phase, which begins within a few hours after surgery, the body activates a strong inflammatory response to manage the tissue injury. Levels of inflammatory molecules (such as TNF-α, IL-1β, and IL-6) rise, immune cells rush to the injury site, and swelling occurs as part of the healing process. 

After this initial surge, the body shifts into the CARS phase to calm the inflammation caused by elevated cytokines and heightened immune activity. However, sometimes, this “calming response” becomes so strong that it results in immunosuppression. This leads to:

  • Reduced immune cell activity (e.g., T cells, B cells & natural killer cells) 
  • Reduced antibody levels (IgG and IgM)
  • Imbalanced protective cytokine levels
  • Impaired neutrophil and macrophage function

These changes typically last from several days to several weeks, depending on the surgery type and patient condition.

Clinical Consequences of Surgery-Induced Immunosuppression

Many post-operative patients, especially those who are older and critically ill, become vulnerable to serious complications due to low immune strength (secondary immunosuppression). Some of the reported clinical complications include: 

For instance, a study found that infants who underwent heart surgery with cardiopulmonary bypass experienced a sharp drop in their IgG levels (reduced to less than half of baseline within 24 hours). More than half of the children (about 53%) developed post-operative hypogammaglobulinemia (low IgG). This shows how significantly surgery can weaken their immune defenses.

Similarly, a prospective study of 60 patients undergoing abdominal surgery found that patients who had emergency surgery developed systemic inflammatory response syndrome (SIRS) after the surgery. The higher SIRS scores were associated with a slower recovery process and longer hospital stays. 

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IVIG and Secondary Immunosuppression After Surgery: What Research Says

There is currently no strong, high-quality data that directly examines the use of IVIG for major surgery-associated secondary immunosuppression. However, several related studies have reported the promising effects of IVIG in managing post-operative complications that may be caused by secondary immunosuppression.  

For instance, a single-center, prospective, double-blind, randomized study reported that when an intravenous immunoglobulin A- and immunoglobulin M-enriched solution was administered immediately after cardiac surgery, it significantly reduced the incidence of post-operative infections in patients.

Similarly, an ICU-based retrospective study found that IVIG use in ICU septic patients significantly reduced in-hospital mortality and improved laboratory biomarkers (e.g., CRP, albumin) over time. 

Also, a recent systematic review and meta-analysis showed that IVIG, particularly IgM-enriched IVIG, is effective in both adult and neonatal sepsis and reduces sepsis mortality. 

These findings collectively indicate that IVIG may help mitigate post-surgical complications that may be induced by immunosuppression. However, more clinical studies and in-depth trials are required to evaluate the benefits of IVIG in secondary immunosuppression after surgery. 

Understanding the Role of IVIG in Surgery-Induced Immunosuppression

Patient receiving IVIG at home while reading a book

As already mentioned above, IVIG shows promise in selected post-operative situations, especially when a patient has severe infection (sepsis/septic shock) due to immunosuppression. Here are several ways in which IVIG can help reduce surgery-induced immunosuppression. 

1. Restores Antibody Levels and Clears Pathogens

It has been found that patients with complex procedures like heart surgery with cardiopulmonary bypass often develop hypogammaglobulinemia (a sharp drop in IgG/IgM levels due to blood loss, catabolism, or capillary leakage). 

Since IVIG is essentially a concentrated pool of antibodies from thousands of donors, administering IVIG can restore antibody levels. These antibodies then fight pathogens that might cause post-operative secondary infections.

2. Modulates Immune Cell Function

Major surgery impairs immune cells, including T cells, B cells, and monocytes, and reduces their ability to respond to infections. IVIG contains antibodies that interact with immune cells to enhance their function:

  • Boosting phagocytosis (how immune cells “eat” bacteria)
  • Supporting T-cell responses
  • Regulating inflammatory pathways

3. Reduces Risk of Post-operative Infections

By restoring antibodies and supporting immune cell function, IVIG may reduce susceptibility to common post-operative infections, such as pneumonia, surgical site infections, or bloodstream infections.

For instance, a retrospective study of adult patients undergoing orthotopic liver transplantation (OLT) who received an IVIG enriched in IgM (rather than a standard IgG-only formulation) had a significantly lower rate of new bacterial or fungal infections in the first 30 days after transplant and better 90-day survival. 

Some studies suggest that patients receiving IVIG show faster normalization of inflammatory markers, indicating improved immune response, even though evidence is limited.

4. Has Potential Anti-Inflammatory Effects

Surgery triggers a two-phase immune response: hyperinflammation (immediately after surgery) followed by immunosuppression. This sudden drop in immune strength also increases the risk of infection. 

IVIG, which has anti-inflammatory properties, can help balance the immune response, prevent excessive inflammation, and support defense against pathogens.

Even though IVIG has these theoretical benefits, current clinical evidence is limited. IVIG is usually reserved for high-risk patients, especially those with documented antibody deficiency, transplant recipients, and patients with severe infections or sepsis risk post-surgery. 

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Who Might Be Considered for IVIG?

IVIG is not routinely used for all surgical patients. Instead, it is generally reserved for selected high-risk individuals based on their clinical status, lab findings, and infection risk. Patients who may be considered include:

  • Solid organ or heart/lung/liver transplant recipients with hypogammaglobulinemia or recurrent/severe infections
  • Post-operative patients with documented low immunoglobulin levels (e.g., low IgG) and a high risk of serious infection
  • Critically ill patients with sepsis or septic shock in whom immune dysfunction or low antibody levels are suspected
  • Infants or other vulnerable patients after complex procedures (such as cardiac surgery with cardiopulmonary bypass) who develop significant antibody loss

The decision to use IVIG is always individualized and made by the treating specialist team, taking into account potential benefits, risks, and alternative therapies.

Risks, Limitations, and Cost

Like any blood-derived product, IVIG carries potential risks and is not appropriate for everyone. Possible side effects include infusion reactions (such as headache, fever, or chills), fluid overload, and, more rarely, serious complications like kidney injury or blood clots in patients who are already at risk.

In addition, the current evidence for IVIG use specifically in surgery-associated secondary immunosuppression is limited and based largely on small or heterogeneous studies, so clear treatment guidelines are still evolving. IVIG is also an expensive therapy and may not be covered or approved for all indications, which means its use is typically reserved for patients in whom the expected benefit justifies the cost and potential risks.

The Bottom Line

Major surgeries can temporarily weaken the immune system, leading to secondary immunosuppression, which increases the risk of infections and delays recovery. IVIG may help boost immunity, but data on its use specifically for surgery-associated secondary immunosuppression are limited. 

Few studies, however, suggest a potential role of IVIG in post-operative complications and indicate that it may be considered in high-risk patients, such as transplant recipients or those with antibody deficiencies or septic shock.

Ingrezza (Valbenazine): Uses, How it Works, Dosage, Side Effects, and More

Published on by Dr. Christine Leduc, PharmD

Ingrezza (pronounced in-GREH-zah) is an oral prescription medication used in adults to manage certain body movement disorders. Its generic name is valbenazine (pronounced val-BEN-a-zeen). It is available as regular capsules to be swallowed whole and as sprinkle capsules that can be opened and mixed with soft food. 

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Ingrezza belongs to the drug class of VMAT2 inhibitors (vesicular monoamine transporter-2 inhibitors) that reduce unwanted or uncontrolled movements. 

The FDA first approved Ingrezza in 2017 for tardive dyskinesia (TD) in adults, and in 2023, it received approval for the treatment of chorea associated with Huntington’s disease. 

Today, this medication is a widely used treatment for people living with movement disorders. This article covers everything you need to know about this drug, including how it works, how to take it, optimal dosage, possible side effects, and important safety information. 

Indications (Approved Uses)

Ingrezza is approved to treat two main conditions in adults, which include:

Tardive Dyskinesia (TD)

Tardive dyskinesia is a nervous system disorder that causes involuntary, uncontrolled muscle movements in the face, tongue, or other body parts. These movements can look like lip-smacking, jaw clenching, tongue rolling, grimacing, twitching, or jerking. 

TD usually develops after the chronic use of antipsychotic medications. 

Chorea From Huntington’s Disease 

Similar to tardive dyskinesia, chorea is another movement disorder that causes sudden, irregular, dance-like, or fidgety movements that a person cannot control. These movements are more common in people with Huntington’s disease (a genetic condition that affects nerve cells in the brain). 

Ingrezza (valbenazine) controls the frequency and severity of uncontrolled movements in patients with tardive dyskinesia and chorea. However, it does not cure these conditions. 

Important Safety Information (Boxed Warning)

Ingrezza may cause serious side effects, including depression and suicidal thoughts or actions. Tell your doctor if you have depression or a history of suicidal thoughts before starting the medication.

Mechanism of Action

Generally, your brain uses a chemical messenger called dopamine to control movement. But when an individual uses antipsychotic drugs chronically, it causes abnormal dopamine signaling in the brain.

Excessive dopamine release leads to repetitive, uncontrollable body movements. Ingrezza helps to reduce the excessive dopamine activity and decrease the involuntary movements seen in TD and chorea. 

Working Mechanism

Valbenazine, the active molecule in Ingrezza, is a vesicular monoamine transporter-2 (VMAT2) inhibitor that selectively and specifically targets VMAT2, a protein that helps regulate the release of dopamine in the brain. 

By blocking the VMAT2 protein, Ingrezza corrects the abnormal dopamine signaling, which results in smoother, more controlled body movements over time. 

Drug Form

Ingrezza comes in two oral forms:

  • Standard capsules that you swallow
  • Sprinkle capsules that may be opened and mixed with soft food for people who have trouble swallowing pills.

Both Ingrezza and Ingrezza Sprinkle capsules come in strengths of 40 mg, 60 mg, and 80 mg.

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Recommended Dosage and Administration

1. Recommended Dosage

A tardive dyskinesia book on a doctor's desk

The FDA-recommended dosage for patients with tardive dyskinesia and Huntington’s chorea is as follows:

  • For Tardive Dyskinesia (TD): The initial dose is 40 mg once a day. After 1 week, increase the dose to the recommended 80 mg once daily.
  • For Huntington’s Chorea: The starting dose is 40 mg once daily, with the dose increased by 20 mg every two weeks until reaching the recommended dose of 80 mg once daily. 

Depending on your response and tolerability to Ingrezza, a dosage of 40 mg or 60 mg once daily may be considered. 

2. Administration Information

Ingrezza capsules and Ingrezza Sprinkle capsules are taken orally once a day, with or without food. 

If you take the capsules, swallow the whole capsule with water. Do not crush or chew. 

If you take Sprinkle capsules:

  • You may open the capsule and sprinkle the contents (granules) over a small amount (1 tablespoonful) of soft food such as applesauce, yogurt, or pudding. 
  • Swallow the soft food without chewing, and drink a glass of water to make sure all of the medicine is swallowed.
  • You may also swallow the Sprinkle capsule whole with water. 
  • Do not add the granules to milk or water.

Possible Side Effects of Ingrezza

Ingrezza capsule and Ingrezza Sprinkle may cause mild or potentially serious side effects. If you experience any of these, consult your healthcare provider.

Mild Side Effects

Some of the mild side effects that you may experience during treatment include:

  • Headache
  • Tiredness and sleepiness (most commonly seen in TD and chorea)
  • Hives (raised itchy red areas) or rash (most commonly seen in people with chorea)
  • Insomnia (most commonly seen in people with chorea)
  • Problems with balance or coordination
  • Dry mouth
  • Restlessness
  • Constipation
  • Blurred vision
  • Weight gain
  • Nausea or vomiting 

In most cases, these side effects are temporary, and some can be easily managed. But if you have ongoing symptoms that bother you, talk to your doctor. 

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Adverse Effects

Though rare, Ingrezza can also cause adverse effects. Some of the serious side effects that have been reported include:

  • Depression and suicidal thoughts: Mostly seen in patients with Huntington’s disease.
  • Severe allergic reactions: Allergic reactions include trouble breathing or shortness of breath; swelling of your face, lips, eyelids, tongue, or throat, or other areas of your skin; trouble with swallowing; rash; or hives.
  • Neuroleptic malignant syndrome (NMS): This is a serious condition that can lead to death. Symptoms include stiff muscles, irregular pulse or blood pressure, increased sweating, high fever, or a very fast or uneven heartbeat. 
  • Heart rhythm problems (QT prolongation): This happens when you take other medicines with Ingrezza. Symptoms may include fast, slow, or uneven heartbeats, lightheadedness, fainting, or shortness of breath.
  • Parkinson’s-like symptoms: This includes tremors, shaking, stiffness, problems with balance or walking.

Your doctor may change your dose, temporarily stop, or permanently stop treatment with Ingrezza if you develop any of the above adverse effects. 

Precautionary Measures 

Before Taking this Medicine

Before taking Ingrezza (valbenazine), tell your doctor if you:

  • Have emotional or mental problems (i.e., depression, nervousness, anxiety, anger, agitation, psychosis, previous suicidal thoughts or suicide attempts) 
  • Have liver problems
  • Have heart disease that is not stable, have heart failure, or have recently had a heart attack
  • Have an irregular heart rhythm or heartbeat (e.g., QT prolongation, heart arrhythmia)
  • Are pregnant or plan to become pregnant (Ingrezza may harm your unborn baby)
  • Are breastfeeding or plan to breastfeed. It is not known if Ingrezza passes into your breast milk. Do not breastfeed during treatment and for 5 days after the final dose
  • Are taking any prescription (e.g., monoamine oxidase inhibitors (MAOI) or digoxin) or over-the-counter medicines, vitamins, and herbal supplements

After Taking This Medicine

Ingrezza is reported to cause slow reaction times (somnolence/sedation). Therefore, it is recommended to avoid driving, operating heavy machinery, or engaging in any potentially dangerous activities until you know how the drug affects you. Dizziness or drowsiness can cause falls, accidents, or severe injuries.

Moreover, avoid taking alcohol or other medicines that can cause sleepiness during treatment. 

Medicines To Avoid While Taking Ingrezza 

You should not take monoamine oxidase inhibitors (MAOIs) with Ingrezza, as these medicines can increase side effects and decrease the effectiveness of Ingrezza. 

Below is the list of drugs that you should avoid taking during your treatment with Ingrezza:

  • MAOIs include phenelzine, tranylcypromine, isocarboxazid, and selegiline
  • Antidepressants like paroxetine or fluoxetine 
  • Antifungal medications, itraconazole and ketoconazole, and antibiotics like clarithromycin
  • Certain seizure medications, such as carbamazepine or phenytoin
  • Antibiotic rifampin
  • Herbal supplements containing St. John’s wort

Moreover, taking Ingrezza with antipsychotics, tricyclic antidepressants, heart medications, and antibiotics may increase the risk of developing irregular heart rhythms.

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Ingrezza Cost

Ingrezza can be expensive for people paying out-of-pocket since it’s a branded drug and is not available in a generic version. However, if you’re new to this drug, you can get a 1-month supply of Ingrezza free of cost through an Ingrezza 30-day free trial program. 

The estimated cost of Ingrezza and Ingrezza Sprinkle (valbenazine) capsules may vary depending on several factors, such as your dosage, what your insurance plan covers, and the type of specialty pharmacy you use. 

The average wholesale price for 30 capsules of Ingrezza (a one-month supply for most patients) is $5,750 for the 40-mg strength and $6,225 for the 80-mg strength. 

On the other hand, if you have commercial (non-government) insurance, you may qualify for a $0 copay through the Ingrezza Savings Card offered by the INBRACE Support Program. Most patients with this type of insurance pay $10 or less per month for their prescription.If you are interested in exploring financial assistance opportunities for Ingrezza, get in touch with one of our specialists for assistance.

IVIG for Hyperimmunoglobulinemia E Syndrome (HIES)

Published on by Dr. Saba Rassouli, PharmD

Hyperimmunoglobulinemia E syndrome (HIES), commonly known as Job’s Syndrome, is a rare primary immunodeficiency disorder where the immune system does not work properly. People with HIES typically have very high levels of immunoglobulin E (IgE) along with frequent infections and skin and lung problems [1][2]. Without proper treatment, HIES can cause permanent organ damage. 

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The good news is that modern medical care has advanced significantly. If you are diagnosed with HIES today, you have far better treatment options than before. In recent years, among the treatment options, intravenous immunoglobulin (IVIG) has shown promising results. In this article, we will discuss everything you need to know about IVIG for hyperimmunoglobulinemia E syndrome (HIES).

Hyperimmunoglobulinemia E Syndrome (HIES): Overview

Normally, your immune system protects you by fighting off foreign pathogens like viruses and bacteria. However, in hyperimmunoglobulinemia E syndrome (HIES) — a rare genetic immune disorder — the immune system does not work properly. As a result, you suffer from frequent infections, especially in your skin and lungs. 

People with HIES have a very high level of an antibody called immunoglobulin E (IgE), but it does not protect them from infections. There are mainly two types of HIES, and they share some common symptoms. However, their complications, treatment needs, and long-term outcomes are different. Below, they are described in detail [1][2]:

  1. Autosomal Dominant HIES (AD-HIES): This type of HIES is usually caused by mutations in the STAT3 gene. You only need to inherit one defective copy of this gene to get this disease. This disease leads to very high immunoglobulin E (IgE) levels, frequent skin and lung infections, and facial, dental, and skeletal abnormalities. 
  2. Autosomal Recessive HIES (AR-HIES): This type of HIES is most commonly caused by DOCK8 gene mutations. Mutations in other genes, such as TYK2, PGM3, and SPINK5, can also cause this disease. You need to inherit the mutated gene from both your parents to get this disease. Compared to AD-HIES patients, AR-HIES patients suffer from more severe viral infections and stronger allergies. 

Signs and Symptoms of HIES

The symptoms of HIES vary from person to person, depending on the type and severity. Some common symptoms include [2][3]:

  • Extremely high levels of an antibody called immunoglobulin E (IgE) in the blood
  • Eczema and skin infections
  • Recurrent lung infections
  • Delayed loss of baby teeth
  • Severe allergies and asthma
  • Facial asymmetry (commonly in AD-HIES)
  • Frequent fungal infections

Treatment Options for HIES

Unfortunately, currently there is no cure for hyperimmunoglobulinemia E syndrome (HIES). The main goals of the current treatment options are to manage your symptoms and prevent infections. Here are some of the standard treatment options for HIES [2][3]:

  • Antibiotics: You need to frequently take antibiotics to treat and prevent bacterial infections.
  • Antiviral Medicines: If you develop infections from viruses, you need to take antiviral medicines. For example, to treat chicken pox, you need to take Aciclovir.
  • Skin Care: Moisturizers, topical steroids, and antiseptic baths help control eczema and prevent skin infections.
  • Surgery: You may need to drain skin and lung abscesses surgically.
  • Intravenous Immunoglobulin (IVIG): IVIG can be a very important supportive care for you. It can help you by boosting your immune function.

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How Does IVIG Help Patients With Hyperimmunoglobulinemia E Syndrome (HIES)?

Patient on bed receiving IVIG at home

Intravenous Immunoglobulin (IVIG) is a purified blood product that contains antibodies from thousands of healthy plasma donors. In HIES, your immune system does not work properly. IVIG can help since it contains ready-made antibodies that help regulate and strengthen your immune system. Here is how IVIG helps patients with HIES:

It Strengthens the Immune System: In HIES, although you have a very high level of IgE, your antibody function is weak. IVIG contains various antibodies, which may help you fight infections more effectively [4].

It Reduces Infection Frequencies: In HIES, the biggest challenge that you face is recurrent skin and lung infections. Several studies showed that IVIG can reduce the frequency of infections [5][7].

It Modulates the Immune System: In HIES, your immune system may sometimes react too strongly, causing eczema, allergies, and severe inflammation. IVIG can calm your immune system by suppressing harmful immune cells and cytokines [6].

It Prevents Lung Damage: HIES can cause repeated lung infections, which can lead to permanent lung damage. IVIG can help prevent long-term lung damage by reducing the frequency of lung infections.

Overall, IVIG is not a cure for HIES. But it plays an important supportive role. If you use it under proper medical supervision, it can improve your quality of life, reduce complications, and prevent long-term organ damage.

Effectiveness of IVIG in Hyperimmunoglobulinemia E Syndrome (HIES): What Studies Say

Because HIES is very rare, large-scale clinical trials are limited. Most of what we know about IVIG for hyperimmunoglobulinemia E syndrome (HIES) comes from small studies and case reports. Although the evidence is limited, these studies suggest that IVIG may help reduce the frequency of infections and improve many immune-related symptoms in HIES patients. 

Over the years, clinical research has produced mixed results. This is partly due to the differences in patient characteristics and disease severity. Some have indicated that IVIG can play a supportive role in managing the condition. For example, a 2007 study presented the case of a 12-year-old boy who suffered from recurrent infections due to HIES. Once IVIG therapy began, he experienced significant clinical improvements and remained free of recurrent infections [5]. Similarly, another study also supports the role of IVIG in treating recurrent infections in HIES [7]. Additionally, some research concluded that IVIG is effective in treating severe eczema and atopic dermatitis in HIES patients by reducing inflammation and stabilizing the immune response [8].

Other reports highlight similar outcomes. A 2001 case report presented the incident of a 37-year-old woman with HIES who suffered from pneumonia. IVIG at a moderate dose improved her pneumonia symptoms [9]. Similarly, another 2001 report presented the case of a 12-month-old infant with severe eczema and pulmonary infection due to HIES. After 35 days of IVIG therapy, the patient showed improvement [10]. 

However, not all findings are positive. In a 1998 study, researchers used high-dose IVIG in one patient with HIES. Their results did not show meaningful clinical improvement in the HIES patient. The researchers observed no significant reduction in IgE levels, IgE synthesis, or other major immune functions [11].

In summary, while we lack large-scale studies, small studies and case reports suggest that IVIG can help some HIES patients — especially those with recurrent infections, severe eczema, or poor response to standard treatments. Results vary from patient to patient, and some studies show little to no benefit. Overall, IVIG is most effective as a supportive therapy rather than a standalone treatment.

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FAQs

Here are some frequently asked questions about IVIG for hyperimmunoglobulinemia E syndrome (HIES).

Does IVIG cure hyperimmunoglobulinemia E syndrome (HIES)?

No. Intravenous Immunoglobulin (IVIG) does not cure HIES. It can reduce the frequency of infections and improve the quality of life.

Is IVIG FDA-approved for treating HIES?

No. IVIG is not FDA-approved for treating HIES. However, it is widely used off-label by doctors to control infections and support immune functions in HIES.

How long does it take for IVIG to work in HIES patients?

The response time varies from patient to patient, depending on the severity of the disease and the patient’s overall health. Many patients begin to notice improvements, such as fewer infections, within a few weeks to a few months of starting IVIG.

LAZCLUZE (Lazertinib) for EGFR-Mutated NSCLC: Uses, How It Works, and Key Facts

Published on by Dr. Christine Leduc, PharmD

Non-small cell lung cancer (NSCLC) is the most common form of lung cancer, affecting over 200,000 people every year in the US [1]. For a long time, chemotherapy has been the main treatment, but it poses many serious side effects. 

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Thanks to advancements in modern medicine, we now have targeted therapies, which have transformed the treatment outcomes for many NSCLC patients. One of the newest targeted therapies is LAZCLUZE (lazertinib). When used together with amivantamab, it offers a powerful treatment option for newly diagnosed patients and improves progression-free survival compared with osimertinib.

If you are researching LAZCLUZE, you may be wondering what it is, how it works, and what the side effects are. In this article, we’ll explore everything you need to know about this medication.

What Is LAZCLUZE?

LAZCLUZE (lazertinib) is an FDA-approved medicine that is used in combination with amivantamab as a first-line treatment for adults with non-small cell lung cancer (NSCLC) that carries specific EGFR gene mutations. The FDA first approved this medicine on August 19, 2024 [3]. This approval was based on extensive clinical data that showed that LAZCLUZE outperformed osimertinib (the previous standard of care) [2].

LAZCLUZE belongs to a group of drugs known as kinase inhibitors. These drugs work by blocking the abnormal signals that help cancer cells grow and spread. LAZCLUZE comes in tablet form. The drug should be taken once daily with or without food. 

What Is It Used To Treat?

LAZCLUZE (lazertinib) is used in combination with amivantamab to treat patients with non-small cell lung cancer (NSCLC) that can not be removed surgically or has spread to other parts of the body (metastatic). The NSCLC must have one of the following mutations on the epidermal growth factor receptor (EGFR) gene [2]:

  • Exon 19 deletions
  • Exon 21 L858R substitution

Before starting the treatment with LAZCLUZE, you must conduct an FDA-approved test to confirm that you have the above-mentioned mutations. 

How Does LAZCLUZE Work?

There is a gene in your body called epidermal growth factor receptor (EGFR), which is responsible for cell growth and multiplication. Normally, these genes would turn on and off when needed to keep cell growth balanced. 

But, in many cases of non-small cell lung cancer (NSCLC), changes (mutations) occur in the EGFR gene. These mutations cause the gene to stay switched on and continuously signal the cancer cells to grow. As a result, cells multiply uncontrollably and form tumors.

LAZCLUZE attaches to the abnormal EGFR receptors inside the cancer cells and blocks the abnormal growth signals. As a result, the growth of the cancer cells slows down [2].

LAZCLUZE is mainly used together with amivantamab. While LAZCLUZE blocks EGFR activity inside the cell, amivantamab targets EGFR and MET on the outside of the cell. If you take them together, they will attack the cancer through multiple pathways. This makes the treatment more effective.

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Side Effects

Patient at home suffering from Lazcluze side effects

Like any other medicine, LAZCLUZE can cause some side effects. While some of these side effects are mild and temporary, others are severe and may require immediate medical attention. Here are some of the side effects [2][4]:

Common Side Effects

  • Rash
  • Nail toxicity
  • Swelling
  • Fatigue
  • Diarrhea
  • Dry skin
  • Pain in the bones, muscles, joints, or nerves
  • Nausea
  • Itching

These side effects are usually mild and go away on their own within a few days. However, if these symptoms do not go away or worsen, contact your doctor.

Serious Side Effects

Serious side effects are rare but can happen. Here are some serious side effects:

Blood Clots: LAZCLUZE can cause blood clots in your legs or lungs, which can be fatal. To prevent this, your doctor will give you anticoagulation medicines for the first 4 months of your treatment.

Serious Allergic Reactions: You may experience allergic reactions while taking LAZCLUZE, which can be severe. Symptoms of serious allergic reactions include fever, increased heart rate, trouble breathing, hives, and swelling of your face, lips, and mouth. 

Lung Problems: This medication can cause serious lung problems, which can be fatal. Symptoms include cough, trouble breathing, and fever. Contact your doctor right away if you experience these symptoms.

Skin Problems: You may experience severe skin problems while taking LAZCLUZE. Symptoms include itching, severe rash, skin redness, and dry skin. It is recommended that you limit your sun exposure during treatment and for 2 months after treatment. Protective clothing and sunscreen can help.

Please note that this is not a complete list of all possible side effects. Contact your doctor immediately if you experience any severe side effects.

Dosing Information of LAZCLUZE

The recommended dose of LAZCLUZE is 240 mg, taken once daily [2]. You can take it with or without food, and it is used together with amivantamab. Your doctor will have you continue taking this medicine until your disease progresses or if there are side effects that are too difficult to manage. If you are taking both medicines on the same day, take LAZCLUZE before the amivantamab infusion. In addition, your doctor will give you anticoagulant (blood-thinning) medicines for the first 4 months of your treatment to prevent blood clots.

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Cost

LAZCLUZE is available in 80 mg and 240 mg tablet form. A pack of 30 tablets (240 mg) costs around $18,350 [5]. However, the actual cost varies depending on your insurance plan, location, and the pharmacy you visit. 

FAQs

What are the clinical benefits of LAZCLUZE?

A large clinical study involving 1,074 patients was conducted to determine the clinical benefits of LAZCLUZE. Patients were given either LAZCLUZE alone, LAZCLUZE plus amivantamab, or osimertinib alone. 

The results showed that patients who received LAZCLUZE plus amivantamab lived for 23.7 months without their condition worsening. In contrast, those who received osimertinib alone went 16.6 months before their condition progressed [2].

Can I use LAZCLUZE during pregnancy or breastfeeding?

No. You should not use LAZCLUZE during pregnancy or breastfeeding because it can harm your unborn baby. You should also avoid breastfeeding for at least 3 weeks after your final dose.

How long do I need to take LAZCLUZE?

You will continue taking this medication until your disease worsens or you experience side effects that are extremely hard to manage.

When to Consider IVIG for Macrophage Activation Syndrome (MAS): Key Insights, Based on the Latest Evidence

Published on by Dr. Robert Hakim, PharmD

Intravenous immunoglobulin (IVIG) for macrophage activation syndrome (MAS) may be an option when patients cannot use other immunosuppressive medications. Discover how IVIG works, learn about its potential benefits, and find answers to your most common questions.

Highlights

  • Macrophage activation syndrome (MAS) is a rare but potentially life-threatening immune response. 
  • IVIG for macrophage activation syndrome may serve as a first- or second-line treatment, depending on the underlying trigger, the patient’s response to other medications, and the stage of macrophage activation. 
  • IVIG is usually preferred when a patient is moderately ill and has an underlying infection. 
  • How IVIG works for MAS is unclear. However, it is thought to work by suppressing macrophage activation and promoting the release of anti-inflammatory molecules. 
  • Large, controlled trials are needed to confirm clear benefits and dosage ranges. 

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A Quick Overview of Macrophage Activation Syndrome (MAS)

MAS is a rare but potentially fatal immune response to rheumatic disorders, cancers, infections, or medications. It occurs when your immune system floods the body with certain types of white blood cells called macrophages and T cells. 

Normally, these cells help destroy disease-causing microorganisms and cancer cells. However, in MAS, these white blood cells become ineffective. As a result, the immune system produces more of these cells, potentially leading to failure of organ systems or death. 

Studies show death may occur in 20% to 53% of cases [1].

What causes the immune system to overreact is not fully understood, and many cases occur without any identifiable triggers. Infections are thought to trigger nearly 50% of MAS cases. Additionally, 10% of children with systemic juvenile idiopathic arthritis develop full-blown MAS [2].

Other triggers can include:

The signs and symptoms of MAS can include:

  • Enlarged liver and spleen
  • Worsening liver function
  • Swollen lymph glands
  • Constantly high fever
  • Seizures and coma
  • Anemia (low red blood cell count)
  • Shock
  • Rash

Treatment of Macrophage Activation Syndrome 

There is no consensus about specific treatment for MAS. 

Before biologics came into existence, IVIG, glucocorticoids, and cyclosporine were widely used to treat rheumatic disease-associated MAS. Currently, these medications are used alone or in combination with biologic agents [3].

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IVIG for Macrophage Activation Syndrome: What We Know So Far

Patient receiving IVIG at home with the assistance of a nurse

IVIG may benefit some patients with MAS, especially when administered early. However, success and efficacy vary widely, and studies have found mixed results. 

For example, in a 2001 study, IVIG produced partial or delayed improvement in five patients but no effects in another five patients. The total number of participants in the study was 20. 

The researchers concluded that IVIG for macrophage activation syndrome might be an option when started during the early stages of macrophage activation [4].

IVIG can be a first-line treatment for patients with chronic granulomatous disease (CGD) complicated by macrophage activation syndrome [5]. 

Researchers from a 2020 case report revealed that IVIG may be [6]:

  • First-line treatment for systemic lupus erythematosus (SLE) with macrophage activation syndrome.
  • Second-line treatment with intravenous steroids. 

Likewise, according to the 2022 EULAR/American College of Rheumatology consensus statements, high-dose glucocorticoids, anakinra with or without IVIG, could be the initial treatment for people with rapidly progressive MAS [7]. 

Available evidence suggests that the effectiveness of IVIG for macrophage activation syndrome is case-dependent. 

For example, IVIG appears to be less effective in severe or refractory cases. However, IVIG therapy should be considered for patients who are unable to receive immunosuppressive medications. IVIG is usually preferred when a patient is moderately ill and has an underlying infection. 

IVIG for Macrophage Activation Syndrome: Understanding the Mechanism of Action

The exact mechanism of action of IVIG for macrophage activation syndrome is poorly understood. 

IVIG is thought to improve MAS symptoms by [8,9]: 

  • Suppressing macrophage activation and release of inflammatory substances.
  • Activating macrophages to release large amounts of anti-inflammatory molecules.
  • Promoting degradation of inflammation-causing macrophages. 

There is no specific dose, which likely depends on the underlying illness and the patient’s response. For moderately ill patients, the usual dose is 1 g to 2 g/kg/dose IV [3].

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Frequently Asked Questions

What are the treatment options for MAS?

Treatment options for MAS are IVIG, glucocorticoids, cyclosporine, plasma exchange, and anakinra. 

What is the difference between MAS and HLH?

Macrophage activation syndrome (MAS) and hemophagocytic lymphohistiocytosis (HLH) are potentially fatal conditions with overlapping symptoms. Nonetheless, MAS is usually associated with rheumatologic disorders, such as systemic juvenile idiopathic arthritis (sJIA), while HLH is often associated with viral infections, cancers, and certain medications [10].

What is MAS in systemic JIA?

MAS is a potentially fatal complication of systemic juvenile idiopathic arthritis (s-JIA). Studies show that 1 in 10 children with s-JIA develops MAS. 

What is the life expectancy in macrophage activation syndrome (MAS)?

In a 2022 study involving 20 adults with rheumatic disease-associated MAS, overall survival at 3 and 6 months was 75.2% and 64.3%, respectively. During the study period, opportunistic infections affected nine participants, and five of them died [11].

What are the FDA-approved IVIG products for macrophage activation syndrome (MAS)?

The FDA has not yet approved any IVIG product for macrophage activation syndrome (MAS). Currently, IVIG use is off-label. In the U.S., the only FDA-approved treatment for MAS is Gamifant (emapalumab-lzsg), which is an interferon gamma-blocking antibody.

Alprolix: A Comprehensive Guide to Uses, Dosage, Side Effects, and Safety

Published on by Dr. Christine Leduc, PharmD

Alprolix injection is a long-acting coagulation factor IX (recombinant) used to treat hemophilia B in children and adults. In this article, you will learn about its mechanism of action, dosage, side effects, cost, and more.

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Before Using Alprolix

Before you receive your first dose of Alprolix for hemophilia B, inform your provider if you: 

  • Are pregnant or planning to get pregnant
  • Are breastfeeding
  • Have proteins called inhibitors, which can stop this medication from working well

Warnings and Precautions

Allergic Reactions

Alprolix injections may cause allergic reactions, which can be fatal. Seek emergency medical care if you experience:

  • Hives
  • Breathing difficulty
  • Swelling in the face or throat

Inhibitor (Antibody) Production

In some cases, your body can produce proteins called “inhibitors” or antibodies, which can prevent this medication from working as intended.

Your healthcare provider will likely order tests to measure Factor IX inhibitors in your blood if:

  • Factor IX level does not increase as expected after starting treatment
  • Bleeding does not stop with an appropriate dose

Complications Due to Blocked Blood Vessels

Factor IX products may increase the risk of a blood vessel being blocked by a circulating blood clot. The risk may be higher if you receive continuous infusions through a large vein. 

Thus, Alprolix is administered as a bolus infusion (rapid injection) over several minutes. 

Alprolix Introduction and Uses

Alprolix injection is a brand-name prescription product. It contains the active ingredient “recombinant coagulation factor IX”, also called eftrenonacog alfa. 

This medication belongs to a class known as coagulation factors or hemostatics. 

A healthcare provider may prescribe this medication for children and adults with hemophilia B to:

  • Control and prevent bleeding episodes
  • Prevent bleeding during surgery
  • Reduce the frequency of bleeding episodes

In people with hemophilia B, blood doesn’t clot properly, as they have a coagulation factor IX deficiency. Factor IX deficiency causes abnormal bleeding after injuries or surgery. Severe hemophilia B may cause spontaneous joint or muscle bleeds. 

Alprolix should not be used to remove inhibitors in people with hemophilia B. 

The US FDA approved Alprolix (then made by Biogen Idec) to treat hemophilia B in 2014. Currently, Bioverativ Therapeutics Inc., a Sanofi Company, makes this medication. 

This medication comes as a sterile white to off-white powder that must be reconstituted into a solution for intravenous (IV) injection. There are three components in an Alprolix kit:

  • One single-dose glass vial, which contains powder of factor IX and the Fc molecule
  • One prefilled syringe containing 5 ml liquid, also called a diluent
  • One reconstitution device (which is used to mix the powder and liquid before administration)

The following strengths are available in the U.S.:

  • 250 IU
  • 500 IU
  • 1,000 IU 
  • 2,000 IU
  • 3,000 IU 
  • 4,000 IU

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Alprolix Mechanism of Action

This medication works by replacing missing coagulation factor IX in people with hemophilia B. It comprises two components:

  1. Factor IX (helps stop bleeds)
  2. Fc Molecule (helps factor IX recirculate in the bloodstream)

This fusion extends Alprolix’s half-life, allowing for less frequent administration. 

Alprolix Dosing 

Before administering Alprolix for hemophilia, your healthcare provider will use a specific formula to determine the dose. Then they will prepare the medication according to the instructions in the package insert.

The dose and duration of the treatment depend on:

  • Age
  • Body weight
  • Severity of factor IX deficiency
  • Desired factor IX level
  • Patient’s condition
  • Location and extent of bleeding

Alprolix Dosing for Control of Bleeding Episodes

Minor and moderate bleeding: The dose is calculated to achieve a factor IX level of 30% – 60% of normal. May be repeated every 48 hours if there is evidence of bleeding. 

Major bleeding: The dose is calculated to achieve a factor IX level of 80% – 100% of normal. May be repeated after 6 – 10 hours, then every 24 hours for 3 days, then every 48 hours until bleeding is resolved.

Alprolix Dosing for Preventing Bleeding During Surgery

Minor surgery: A single infusion to achieve a factor IX level of 50 – 80 IU/dL. Repeat after 24 – 48 hours until bleeding stops.

Major surgery: Initial infusion to achieve a factor IX level of 60 – 100 IU/dL. May be repeated after 6 – 10 hours and then every 24 hours for the first 3 days, then every 48 hours until bleeding stops.

Alprolix Dosing for Routine Prevention of Bleeding Episodes

The typical starting doses are:

  • For individuals 12 years or older: 50 IU/kg once weekly or 100 IU/kg every 10 days
  • For individuals up to 11 years: 60 IU/kg once weekly

Children younger than 12 years may need more frequent or higher doses. For individuals 12 years or older, dose adjustment is not usually necessary.

Alprolix Proper Use

  • Use this or any medication exactly as directed. 
  • You will likely receive your first dose in a hospital or clinic setting. 
  • Then, your healthcare provider will teach you how to prepare and self-administer this medication. 
  • Ask your doctor or pharmacist if you have problems understanding any part of “Instruction for Use.”

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Alprolix Storage and Handling

Before Preparing the Solution

  • Store in the original package to prevent exposure to sunlight.
  • Maintain storage temperature between 2°C and 8°C (36°F and 46°F). Avoid freezing.
  • You may store this medication at room temperature (maximum temperature of 30°C or 86°F) for up to 6 months. The expiration date should be printed on the label.
  • If you store this medication at room temperature, record the date when it was removed from refrigeration.
  • Once at room temperature, avoid returning the product to the refrigerator.
  • Never use Alprolix beyond the expiration date printed on the vial or 6 months after storage at room temperature.

After Preparing the Solution

  • You may store the solution at room temperature (maximum temperature 30°C or 86°F) for up to 3 hours. Avoid exposure to direct sunlight.
  • Discard the solution if not used within 3 hours after reconstitution.
  • Never use a solution that is cloudy or has particulate matter.
  • Throw away any unused product.

Alprolix Side Effects

Boy suffering from a headache as a side effect of Alprolix

Side effects can be mild or severe. 

The most common side effects in previously treated individuals can include:

  • Headache
  • Tingling or burning in the mouth
  • Dizziness
  • Fatigue
  • Decreased appetite
  • Low blood pressure
  • Obstructed urine flow

The most common side effects in previously untreated individuals can include:

  • Inhibitor (antibody) production
  • Pain, redness, or itching at the injection site
  • Allergy

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Alprolix Cost

Cost can vary depending on your insurance plan, location, and pharmacy. Contact your insurance provider to find out if your plan covers this medication or if you need prior authorization.

Sanofi, the manufacturer of Alprolix, offers three financial assistance programs to help you access treatment:

Alprolix Free Trial Plus Program: With this program, you may be able to get a FREE 30-day supply with a valid prescription from your healthcare provider.

Alprolix Factor Access Program: This program can help you access treatment even if your insurance is interrupted.

Alprolix Sanofi Copay Program: Contact a Sanofi case manager at 1-833-723-5463.

To explore these programs or apply for copay assistance, contact our team at AmeriPharma for financial assistance.

IVIG for Hematophagocytic Lymphohistiocytosis (HLH)

Published on by Dr. Saba Rassouli, PharmD

Hematophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition where the immune system becomes dangerously overactive, attacking its own tissues and organs. Without early and proper treatment, HLH can lead to severe inflammation, multi-organ failure, and even death. Although HLH primarily affects infants and young children, adults can also develop this condition [2].

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To improve survival in HLH, proper and early treatment is key. In recent years, among the available treatment options, intravenous immunoglobulin (IVIG) therapy has delivered promising results. In this article, we will discuss everything you need to know about IVIG for hematophagocytic lymphohistiocytosis (HLH).

Hematophagocytic Lymphohistiocytosis (HLH): Overview

Normally, when a foreign pathogen like a virus or bacteria enters the body, the immune system launches a controlled response to eliminate the infection. When the foreign pathogen is destroyed, the immune response gradually fades. However, in hematophagocytic lymphohistiocytosis (HLH), the immune system becomes overactive. Various immune cells, such as T-lymphocytes and macrophages, start attacking the blood cells, tissues, and organs. This can cause multiple organ failure and lead to death [2][3].

There are two main types of HLH. They are [1]:

  1. Primary (Familial) HLH: An inherited disease caused by genetic mutations. It usually appears in infancy or early childhood. Primary HLH accounts for about 25% of all HLH cases.
  1. Secondary (Acquired) HLH: Secondary HLH is triggered by various causes, including infections (such as Epstein-Barr virus), autoimmune diseases (like lupus), cancers, or certain medications.

Signs and Symptoms of HLH

The symptoms of HLH develop rapidly, and the severity varies from person to person. Some common symptoms of HLH include [1][2]:

  • High and persistent fever
  • Enlarged liver and spleen
  • Low blood cell counts (anemia, low platelets, low white blood cells)
  • Skin rash
  • Enlarged lymph nodes
  • Elevated iron level in blood
  • Jaundice (yellowing of the skin or eyes)

Standard Treatment for HLH

Doctor discusing HLH with a patient at a hospital

The main goal of HLH treatment is to suppress the overactive immune system and prevent further organ damage. Standard treatment depends on whether HLH is primary or secondary and may include [1][3]:

  • Corticosteroids (e.g., dexamethasone): These drugs reduce inflammation and calm the overactive immune system.
  • Etoposide: A chemotherapy drug used to destroy overactive immune cells.
  • Hematopoietic Stem Cell Transplant: This is the only possible cure for primary HLH.
  • Supportive care: Includes antibiotics, antivirals, blood transfusions, and management of organ failure.
  • Intravenous Immunoglobulin (IVIG): IVIG, when combined with other medications such as corticosteroids, is an effective treatment for HLH. It can help you get better by reducing the inflammation and regulating the immune response.

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How Does IVIG Help Patients With Hematophagocytic Lymphohistiocytosis (HLH)?

Intravenous Immunoglobulin (IVIG) is a highly purified medical product made from the plasma of thousands of healthy blood donors. It contains antibodies that help balance and regulate the immune system in various autoimmune, inflammatory, or immunodeficiency conditions. 

In hematophagocytic lymphohistiocytosis (HLH), the immune system becomes hyperactive and attacks its own blood cells, tissues, and organs. In this condition, IVIG can help by:

Modulating the Immune Response: IVIG can help calm an overactive immune system by suppressing immune cells like T-cells and macrophages that cause inflammation and tissue damage [4][5]. By regulating these cells, IVIG helps prevent further damage to the body.

Neutralizing Cytokines and Autoantibodies: In HLH, the immune system releases excessive cytokines (molecules that cause widespread inflammation) and produces autoantibodies that attack the body’s own cells. This uncontrolled immune activity leads to severe tissue damage and organ failure. IVIG can reduce inflammation and protect the body from further harm by neutralizing these harmful cytokines and autoantibodies [6].

Providing Passive Immunity: In cases where HLH is triggered by an infection, IVIG offers ready-made antibodies that help the body fight off the infection. 

Protecting Organs: By reducing the inflammatory response, IVIG helps protect vital organs from further damage. 

Overall, IVIG does not cure HLH on its own. But it serves an essential supportive role, especially in secondary HLH caused by infections or autoimmune diseases. When used early and alongside other standard treatments, IVIG can help stabilize patients, control inflammation, and improve overall outcomes [7].

Effectiveness of IVIG in Hematophagocytic Lymphohistiocytosis (HLH): What Studies Say

Studies suggest that the effectiveness of Intravenous Immunoglobulin (IVIG) in treating Hemophagocytic Lymphohistiocytosis (HLH) largely depends on the type of HLH and the timing of treatment. While IVIG is not a cure for HLH, it is a valuable tool, particularly in secondary HLH (sHLH), which develops as a result of infections, autoimmune diseases, or cancers.

Over the past decade, research has increasingly supported IVIG combined with corticosteroids as an initial treatment for secondary HLH (sHLH). This approach is especially valuable in patients for whom doctors want to avoid the serious side effects associated with etoposide, a chemotherapy drug commonly used in standard HLH treatment regimens. 

For example, a 2014 study involving 40 children with HLH found that the combination of IVIG and Dexamethasone was a more preferable initial regimen than etoposide-based treatment. This is because the IVIG regimen can avoid the long-term risks of secondary cancers linked to etoposide use [8].

A more recent 2024 study highlighted the critical importance of early administration. The study observed that secondary HLH (sHLH) patients with sepsis who received IVIG and Dexamethasone early in their disease course showed significant clinical and laboratory improvements. On the other hand, patients who received IVIG late in their treatment had poorer outcomes, often resulting in death. This study suggests that there is a narrow window for maximum efficacy [7].

Similarly, the UK’s National Health Service (NHS) includes IVIG as a recognized treatment option for secondary HLH [10]. In one reported case study, two secondary HLH patients were treated with IVIG for five consecutive days [9]. Both of them recovered fully from their illness.

However, IVIG’s effectiveness appears to diminish in other HLH subtypes. A study of 46 adult HLH patients reported better long-term survival in infection-associated HLH but very poor long-term survival in malignancy-associated HLH [11]. Again, in cases of primary HLH, etoposide-based chemotherapy remains the standard of care. 

In summary, IVIG plays a valuable supportive role in managing infection-triggered or autoimmune-related secondary HLH when given early in the disease course. However, in primary HLH or cancer-related secondary HLH, the benefits of IVIG are limited. Therefore, we need more large-scale clinical trials to better define IVIG’s role, optimal dosing, and combination strategies in different subtypes of HLH.

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FAQs

Here are some of the most frequently asked questions about IVIG for hematophagocytic lymphohistiocytosis (HLH):

1. Is IVIG FDA-approved for treating HLH?

No. IVIG is not FDA-approved specifically for the treatment of HLH. However, IVIG is used “off-label” for treating HLH based on clinical judgment. Studies showed that IVIG is effective in treating HLH, particularly infection-associated or immune-related HLH.

2. Can IVIG cure HLH on its own?

No. IVIG alone cannot cure HLH. It can help stabilize the immune system and reduce inflammation. But IVIG usually needs to be combined with corticosteroids (e.g., dexamethasone) or other therapies. 

3. How long does it take for IVIG to work in HLH?

Some patients see clinical improvements within 24-72 hours after starting IVIG treatment. However, the results vary depending on the severity, cause, and timing of treatment.

4. What is the typical dose of IVIG for HLH?

The typical dose of IVIG ranges from 1 to 2 grams per kilogram of your body weight. It can be given as a single infusion or divided into smaller doses spread over 2 – 5 days [12][13][14]. The exact dosing schedule depends on several factors, including the severity of your condition, response to treatment, and how well you tolerate the infusion. Your doctor will determine the most suitable dose and duration for you to achieve the best results.