Understanding hemolysis

  • still had
    anemia*

  • still had
    fatigue

  • still needed a
    transfusion*

*These data are from a retrospective study of 141 people who had been treated with eculizumab in the Leeds Center of the UK PNH National Service. A retrospective study is a type of study that takes data from previous studies and analyzes it. It is different from other studies that are designed and run to collect new data.

These data are from a survey (a collection of information from individuals through response to questions) of 58 people ≥18 years of age who were treated with eculizumab or ravulizumab in the US. They self-reported a PNH diagnosis and were recruited through a patient advocacy group in July 2020. The survey is ongoing.

In general, prospective studies designed to collect new data are considered scientifically stronger than retrospective or survey studies.

Starting a conversation with your doctor about PNH symptoms may help you better manage your health. This questionnaire can help explain how you’re feeling.

If you want to learn more about ongoing hemolysis or share this science with others, download our brochure.

There are 2 types of hemolysis, intravascular (IVH) and extravascular (EVH), both of which may be responsible for ongoing PNH symptoms. The science behind the complement systemcomplement systemThe complement system, also referred to as the complement cascade, is an army of more than 50 different proteins that work together to get rid of dangerous cells and fight infection. and ongoing hemolysishemolysisThe “breaking apart” of red blood cells. It can occur when the immune system attacks these cells as though they were dangerous viruses or bacteria. When red blood cells break open, hemoglobin is released. Hemolysis causes many of the symptoms of PNH. may explain why these ongoing PNH symptoms can occur even with treatment

Two key proteins in the complement system are known as C3c3A protein located in the blood that acts as a central point of the complement system, regulating its activation. and C5c5A protein located in the blood that plays an important role in inflammation and intravascular hemolysis caused by the complement system.

C3 and C5 proteins are activated in a specific order as your immune system detects viruses, bacteria, or damaged cells. Like dominoes falling, each activation triggers another activation in a series of steps known as a “cascade”

There are 2 types of hemolysis, intravascular (IVH) and extravascular (EVH), both of which may be responsible for ongoing PNH symptoms. The science behind the complement systemcomplement systemThe complement system, also referred to as the complement cascade, is an army of more than 50 different proteins that work together to get rid of dangerous cells and fight infection. and ongoing hemolysishemolysisThe “breaking apart” of red blood cells. It can occur when the immune system attacks these cells as though they were dangerous viruses or bacteria. When red blood cells break open, hemoglobin is released. Hemolysis causes many of the symptoms of PNH. may explain why these ongoing PNH symptoms can occur even with treatment

Two key proteins in the complement system are known as C3c3A protein located in the blood that acts as a central point of the complement system, regulating its activation. and C5c5A protein located in the blood that plays an important role in inflammation and intravascular hemolysis caused by the complement system.

C3 is activated, then "kicks off" the rest of the cascade by splitting into C3a and C3b

C3b activates C5

Extravascular hemolysis occurs when red blood cells are destroyed in the liver and spleen

C5 splits into C5a and C5b

C5b is one of the proteins that forms the membrane attack complex (MAC)membrane attack complex (MAC)A structure that is formed within the chain of complement system activation. It also attaches to cells which the body believes are threatening and helps destroy them.. The MAC helps destroy dangerous cells, like viruses and bacteria, in the body.

Intravascular hemolysis attack occurs when red blood cells are destroyed inside the blood vessels by the MAC.

Both IVH and EVH can occur in your body at the same time and cause the PNH symptoms you experience

IVH is an attack that occurs when PNH red blood cells are destroyed inside the blood vessels

C3 proteins bind to PNH red blood cells, which "tag" them for destruction

This tagging sets off a chain of events within the complement system

C3 splits into 2 other proteins, C3a and C3b

C3b triggers the C5 protein

The C5 protein splits into 2 other proteins, C5a and C5b

C5b helps form the MAC. The MAC creates holes in the PNH red blood cells, causing them to burst

An EVH attack occurs when PNH red blood cells are destroyed in the liver and spleen

PNH red blood cells that are not destroyed from IVH continue to be covered in C3 proteins. This is known as C3-loading

The C3-loaded PNH red blood cells are destroyed in the liver and spleen by special cells called macrophages

There are 2 types of hemolysis, intravascular (IVH) and extravascular (EVH), both of which may be responsible for ongoing PNH symptoms. The science behind the complement systemcomplement systemThe complement system, also referred to as the complement cascade, is an army of more than 50 different proteins that work together to get rid of dangerous cells and fight infection. and ongoing hemolysishemolysisThe “breaking apart” of red blood cells. It can occur when the immune system attacks these cells as though they were dangerous viruses or bacteria. When red blood cells break open, hemoglobin is released. Hemolysis causes many of the symptoms of PNH. may explain why these ongoing PNH symptoms can occur even with treatment

Two key proteins in the complement system are known as C3c3A protein located in the blood that acts as a central point of the complement system, regulating its activation. and C5c5A protein located in the blood that plays an important role in inflammation and intravascular hemolysis caused by the complement system.

C3 and C5 proteins are activated in a specific order as your immune system detects viruses, bacteria, or damaged cells. Like dominoes falling, each activation triggers another activation in a series of steps known as a “cascade”

Both IVH and EVH can occur in your body at the same time and cause the PNH symptoms you experience

Intravascular hemolysis attack occurs when red blood cells are destroyed inside the blood vessels by the MAC.

An EVH attack occurs when PNH red blood cells are destroyed in the liver and spleen

C3 proteins bind to PNH red blood cells, which "tag" them for destruction

This tagging sets off a chain of events within the complement system

An EVH attack occurs when PNH red blood cells are destroyed in the liver and spleen
An EVH attack occurs when PNH red blood cells are destroyed in the liver and spleen

The C3-loaded PNH red blood cells are destroyed in the liver and spleen by special cells called macrophages

Both IVH and EVH can occur in the body at the same time and cause the PNH symptoms you experience

An EVH attack occurs when PNH red blood cells are destroyed in the liver and spleen

PNH red blood cells that are not destroyed from IVH continue to be covered in C3 proteins. This is known as C3-loading

The C3-loaded PNH red blood cells are destroyed in the liver and spleen by special cells called macrophages

To address ongoing PNH symptoms, it may be important to control both IVH and EVH

Both IVH and EVH can occur in your body at the same time and cause the PNH symptoms you experience

IVH is an attack that occurs when PNH red blood cells are destroyed inside the blood vessels by the MAC.

test
C3 and C5 proteins are activated in a specific order as your immune system detects viruses, bacteria, or damaged cells. Like dominoes falling, each activation triggers another activation in a series of steps known as a “cascade”

Both IVH and EVH can occur in your body at the same time and cause the PNH symptoms you experience

Two key proteins in the complement system are known as C3c3A protein located in the blood that acts as a central point of the complement system, regulating its activation. and C5c5A protein located in the blood that plays an important role in inflammation and intravascular hemolysis caused by the complement system.

test

C3 and C5 proteins are activated in a specific order as your immune system detects viruses, bacteria, or damaged cells. Like dominoes falling, each activation triggers another activation in a series of steps known as a “cascade”

Your lab values and symptoms may help determine if you have ongoing hemolysis

  • Below normal levels of HEMOGLOBIN (Hb)
  • Elevated lactate dehydrogenase (LDH)
  • elevated reticulocyte count and/or
  • elevated levels of bilirubin

Every person’s experience with this disease can be unique. Your doctor will consider a variety of test results and all your signs and symptoms when monitoring your PNH. It’s always best to speak to your doctor about your test results and what they may mean for your health