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Managing Cold Agglutinin Disease Today

Improving understanding of cold agglutinin disease

Cold Agglutinin Disease

Cold agglutinin disease (CAgD) is a rare disease, affecting ~16 out of every one million people1. There are an estimated 10,000 people in the U.S. and Europe living with CAgD.

Together with doctors, researchers, and those who are living with the disease, we are working to improve our understanding of CAgD and to share that knowledge with the people impacted by it.


Understanding Cold Agglutinin Disease

CAgD is a severe, chronic, blood disorder that results in the immune system mistakenly destroying red blood cells.

People with CAgD suffer from chronic anemia due to the high rate of red blood cell destruction happening as part of the disease.

Anemia can be severe for people with CAgD, and result in symptoms including crippling fatigue, shortness of breath, weakness, lightheadedness, a fast heartbeat, or even chest pain. 2

Cold agglutinin disease can occur suddenly or gradually, and people with the rare blood disease can also experience bluish or pale color in their fingers, toes, nose, ears or face along with numbness or tingling.

Symptoms of CAgD can be made worse by infections or inflammation resulting in more severe anemia.

Cold agglutinin disease is an autoimmune disorder in which low-grade lymphoproliferative cells abnormally activate the classical complement pathway (a part of the body’s immune system). This complement activation results in the destruction of a patient’s red blood cells causing anemia and other complications.

CAgD is a form of autoimmune hemolytic anemia.

Autoimmune: person’s immune system is attacking his/her own body
Hemolytic: blood
Anemia: not enough healthy red blood cells

Diagnosing Cold Agglutinin Disease

Diagnosis of CAgD includes a physical exam, blood tests and a medical history review. However, because CAgD is rare, it can take a while from the time people with CAgD first notice symptoms to the time they receive a diagnosis.

Living with Cold Agglutinin Disease

People living with CAgD typically cycle through periods of more severe anemia, followed by times when their anemia may be more mild or moderate. The length of time between these cycles is unpredictable and can vary tremendously—from weeks to even years.

In one study, most patients experienced 7 severe anemia events per year.1

In addition to anemia and the symptoms that come with it, people living with CAgD are more likely to experience blood clots in either the veins or arteries, which can have serious consequences. Stroke and heart attack are examples of blood clots in the arteries; deep vein thrombosis (DVT) and pulmonary embolism are examples of blood clots in the veins.

These thromboembolic events can be life threatening, and researchers are beginning to better understand the true connection between CAgD and these types of events.

A new retrospective study found that people with CAgD had a 55% overall increased rate of thromboembolic events than people without CAgD.4

The same study showed that the risk of a thromboembolic event was linked to the presence of hemolysis (red blood cell destruction), which is constantly happening in people with CAgD, not the severity of a person’s anemia.4

Managing Cold Agglutinin Disease Today

Today, there are no medicines approved to treat CAgD. Instead, doctors use a few different treatments – sometimes in combination – to treat the anemia and the symptoms that most bother their patients1. Blood transfusions are often used to treat severe, symptomatic anemia3, and most people with CAgD currently require blood transfusions at some point1.

A recent retrospective study showed an average of 11 transfusions per patient per year.1

There is a strong need for approved medicines that can act quickly, stop the hemolysis, and improve the anemia for people living with CAgD. To learn about our clinical trials underway, click here.

BIVV009 is an investigational candidate in development for cold agglutinin disease that is currently in Phase 3 clinical development. It has received breakthrough therapy designation by the U.S. Food and Drug Administration (FDA), and orphan drug designation by the FDA and European Medicines Agency.

What Can I Do?

If you are currently living with CAgD or have symptoms, you should talk to your doctor. It may also be helpful to consider an appointment to see a doctor who specializes in blood disorders, such as a hematologist. You might also ask your doctor if there are any clinical trials for people with CAgD that would be appropriate for you.

You could also:

Join a support group – There are online support groups where people with CAgD and their family members can share stories, ideas, helpful tips, and other useful information. These include:

Read more about CAgD – Learn as much as you can about CAgD so that you can take more control of your own care, and explain it to others, like doctors, nurses, family, and friends. These websites can help get you started:

Call Bioverativ – Let us help connect you with resources for cold agglutinin disease:
1-833-CAD-CHAT (1-833-223-2428)



  1. Mullins M, et al. Cold agglutinin disease burden: a longitudinal analysis of anemia, medications, transfusions, and health care utilization. Blood Advances. 2017;1(13):839-848
  2. Cook K. Anemias. In: DiPiro JT, et al., eds. Pharmacotherapy: A Pathophysiologic Approach. 10th ed. New York, NY: McGraw-Hill; 2017:1593-1610.
  3. Packman CH. Hemolytic anemia resulting from immune injury. In: Kaushansky K, et al, eds. Williams Hematology. 9th ed. New York, NY: McGraw-Hill; 2016:823-845.
  4. Broome, C., et al. Incidence of Thromboembolic Events Is Increased in a Retrospective Analysis of a Large Cold Agglutinin Disease (CAD) Cohort. 59th Annual Meeting of the American Society of Hematology; December 9-12, 2017; Atlanta.