What is Primary HLH? | Primary HLH | Gamifant Cares™

Father and 2 Kids

Understanding
primary HLH

Finding out that you or your loved one have primary HLH can be overwhelming. The following information is for you and your family to learn about primary HLH, its symptoms, and what you might expect after diagnosis.

What is primary HLH?

Primary HLH is a rare genetic condition, which means it can be passed down in families. It usually affects babies and young children, but it can affect older children and adults as well.

Primary HLH is caused by a genetic mutation that keeps the immune system from working properly.

When the immune system is working properly, it finds and attacks foreign invaders like viruses and bacteria to protect the body
In people with primary HLH, the immune system doesn’t work right. It attacks a person’s own cells and organs instead

What does pHLH stand for?

primary hemo phagocytic lympho histiocytosis

genetic/inherited(also called familial)

blood

cells that eat

relating to the lymphatic system

an excess of macrophages (a type of white blood cell)

Here's how to pronounce primary HLH

What is the difference between primary and secondary HLH?

While researchers are still working to fully understand HLH, so far it is known to exist in 2 main types: primary and secondary.

Primary HLH could be inherited, which means it is passed down from a parent to a child.

Secondary HLH could be triggered by other medical problems, such as an infection or a disease like cancer.

Although the symptoms for both may be similar, it is thought that each has a different cause and may require different approaches to treatment.

Care Tip

To understand more about your or your loved one’s condition, it’s important to know what happens in the body of someone with primary HLH and what some of the symptoms of primary HLH can be.

Please be sure to speak with your doctor about HLH, including any symptoms.

WHAT HAPPENS INSIDE THE BODY?

See how primary HLH works and what happens inside the body

WHAT HAPPENS INSIDE THE BODY?

See how primary HLH works and what happens inside the body

What is GAMIFANT?

Gamifant (emapalumab-lzsg) is a prescription medicine used for the treatment of hemophagocytic lymphohistiocytosis (HLH) in adults and children (newborn and older) with:

primary HLH whose disease has come back or progressed,

Important Safety Information

Gamifant can cause serious side effects including:

Infections. GAMIFANT affects your immune system and may lower the ability of your immune system to fight infections. Gamifant may increase your risk of serious

What is GAMIFANT?

Gamifant (emapalumab-lzsg) is a prescription medicine used for the treatment of hemophagocytic lymphohistiocytosis (HLH) in adults and children (newborn and older) with:

primary HLH whose disease has come back or progressed, other medicines have not worked well enough or cannot be tolerated.
HLH/macrophage activation syndrome (MAS) in Still’s disease, including systemic Juvenile Idiopathic Arthritis (sJIA), when glucocorticoids have not worked well enough or cannot be tolerated, or with MAS that has come back.

Important Safety Information

Gamifant can cause serious side effects including:

Infections. GAMIFANT affects your immune system and may lower the ability of your immune system to fight infections. Gamifant may increase your risk of serious infections that can lead to death. These infections include tuberculosis (TB), histoplasmosis, herpes zoster infection (shingles), and other infections caused by viruses, fungi, or bacteria that can spread throughout the body. Infections are common in people treated with Gamifant. Your healthcare provider will:
- Test you for TB before you start Gamifant
- Treat you with a medicine for TB if you are at risk for TB or if you have a known positive TB test

Before starting Gamifant, tell your doctor if you:

had TB in the past, or if you or a member of your family have been in recent close contact with someone with TB
have ever had a positive TB skin test (PPD)
currently have or have had a history of infections, including histoplasmosis or herpes zoster (shingles)
are being treated for an active infection
have symptoms of an infection, such as fever, sweat and chills, cough, breathing problems, blood in mucus (phlegm), or warm, red, or painful skin or sores on your body

After starting Gamifant, tell your healthcare provider if:

New symptoms of an infection appear
Symptoms of an infection that you already had when starting Gamifant worsen. Your healthcare provider will monitor you closely for signs and symptoms of infections during treatment with Gamifant

Your healthcare provider may give you medicine to help prevent certain infections before you receive Gamifant and will monitor you closely for signs and symptoms of infections during treatment with Gamifant.

Infusion Reactions. These are common, can also be severe, and can happen during or shortly after treatment with Gamifant. Your healthcare provider may temporarily stop your infusion and treat your symptoms before continuing your infusion if you have severe infusion reactions. Tell your healthcare provider right away if you get any of the following symptoms:

skin redness
itching
fever
rash
excessive sweating
headache
bone pain

chills
chest pain
shortness of breath
nausea or vomiting
lightheadedness or dizziness
tingling, burning, or numbness
cold hands, arms, legs, or feet

Before starting Gamifant, tell your doctor about all of your medical conditions including if you:

have an infection
have received or are scheduled to receive an immunization (vaccine). You should not receive live or live attenuated vaccines while taking Gamifant and for at least 4 weeks after your last dose of Gamifant
are pregnant or plan to become pregnant
are breastfeeding or plan to breastfeed

Tell your healthcare provider about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements.

The most common side effects of Gamifant in people with primary HLH include: infections, high blood pressure (hypertension), and fever.

The most common side effects of Gamifant in people with HLH/MAS in Still’s disease include: viral infection and rash.

These are not all the possible side effects of Gamifant. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

Please see Patient Medication Guide for Gamifant.

What is GAMIFANT?

Gamifant (emapalumab-lzsg) is a prescription medicine used for the treatment of hemophagocytic lymphohistiocytosis (HLH) in adults and children (newborn and older) with:

primary HLH whose disease has come back or progressed,

Important Safety Information

Gamifant can cause serious side effects including:

Infections. GAMIFANT affects your immune system and may lower the ability of your immune system to fight infections. Gamifant may increase your risk of serious

What is GAMIFANT?

Gamifant (emapalumab-lzsg) is a prescription medicine used for the treatment of hemophagocytic lymphohistiocytosis (HLH) in adults and children (newborn and older) with:

primary HLH whose disease has come back or progressed, other medicines have not worked well enough or cannot be tolerated.
HLH/macrophage activation syndrome (MAS) in Still’s disease, including systemic Juvenile Idiopathic Arthritis (sJIA), when glucocorticoids have not worked well enough or cannot be tolerated, or with MAS that has come back.

Important Safety Information

Gamifant can cause serious side effects including:

Infections. GAMIFANT affects your immune system and may lower the ability of your immune system to fight infections. Gamifant may increase your risk of serious infections that can lead to death. These infections include tuberculosis (TB), histoplasmosis, herpes zoster infection (shingles), and other infections caused by viruses, fungi, or bacteria that can spread throughout the body. Infections are common in people treated with Gamifant. Your healthcare provider will:
- Test you for TB before you start Gamifant
- Treat you with a medicine for TB if you are at risk for TB or if you have a known positive TB test

Before starting Gamifant, tell your doctor if you:

had TB in the past, or if you or a member of your family have been in recent close contact with someone with TB
have ever had a positive TB skin test (PPD)
currently have or have had a history of infections, including histoplasmosis or herpes zoster (shingles)
are being treated for an active infection
have symptoms of an infection, such as fever, sweat and chills, cough, breathing problems, blood in mucus (phlegm), or warm, red, or painful skin or sores on your body

After starting Gamifant, tell your healthcare provider if:

New symptoms of an infection appear
Symptoms of an infection that you already had when starting Gamifant worsen. Your healthcare provider will monitor you closely for signs and symptoms of infections during treatment with Gamifant

Your healthcare provider may give you medicine to help prevent certain infections before you receive Gamifant and will monitor you closely for signs and symptoms of infections during treatment with Gamifant.

Infusion Reactions. These are common, can also be severe, and can happen during or shortly after treatment with Gamifant. Your healthcare provider may temporarily stop your infusion and treat your symptoms before continuing your infusion if you have severe infusion reactions. Tell your healthcare provider right away if you get any of the following symptoms:

skin redness
itching
fever
rash
excessive sweating
headache
bone pain

chills
chest pain
shortness of breath
nausea or vomiting
lightheadedness or dizziness
tingling, burning, or numbness
cold hands, arms, legs, or feet

Before starting Gamifant, tell your doctor about all of your medical conditions including if you:

have an infection
have received or are scheduled to receive an immunization (vaccine). You should not receive live or live attenuated vaccines while taking Gamifant and for at least 4 weeks after your last dose of Gamifant
are pregnant or plan to become pregnant
are breastfeeding or plan to breastfeed

Tell your healthcare provider about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements.

The most common side effects of Gamifant in people with primary HLH include: infections, high blood pressure (hypertension), and fever.

The most common side effects of Gamifant in people with HLH/MAS in Still’s disease include: viral infection and rash.

These are not all the possible side effects of Gamifant. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

Please see Patient Medication Guide for Gamifant.