
What is a cold agglutinin test and how is it done?
How Is the Test Done? This is a blood test that measures the amount of cold agglutinins in your blood. The test may also be done while the blood sample is exposed to different temperatures; this helps the doctor figure out at what temperature your red blood cells start to clump together. There is nothing you need to do in advance to prepare.
Do you have cold agglutinin disease?
But you might have more reasons for not liking cold temperatures if you have a rare condition known as “ cold agglutinin disease .” Cold agglutinins are antibodies that, instead of helping to fight bad things like bacteria in your body, attack your red blood cells.
What is cold agglutinin disease (cold antibody hemolytic anemia)?
It’s also called cold antibody hemolytic anemia. About 1 person in 300,000 gets cold agglutinin disease. It shows up most often in people over 60, and women are more likely to get it than men. When you breathe in, your red blood cells carry oxygen from your lungs to the rest of your body.

Is cold agglutinin serious?
CAD can lead to severe and even life-threatening heart conditions in emergency scenarios. Related to its name — “cold” agglutinin disease — CAD causes most intense symptoms in cold climates or chilly weather.
How long can you live with cold agglutinin disease?
One Norwegian study reported the median age of primary CAD patients to be 76 years, with a median age of onset of 67 years, a median survival of about 12.5 years following diagnosis, and a median age of 82 years at death.
Which of these underlying conditions can be associated with cold agglutinin disease?
Causes of polyclonal secondary cold agglutinin disease include the following: Mycoplasma infections - M pneumoniae. Infectious mononucleosis due to Epstein-Barr virus (EBV) or cytomegalovirus (CMV) Other viral infections - Mumps, varicella, rubella, adenovirus, HIV, influenza, hepatitis C.
What is the cure for cold agglutinin?
The only drug approved to specifically treate CAD is sutimlimab-jome (Enjaymo). It works by inhibiting the destruction of red blood cells and so decreases the need for blood transfusions. Another drug commonly used for CAD is rituximab, a drug that targets certain immune cells.
What type of doctor treats cold agglutinin disease?
Hematologists. Hematologists specialize in the treatment of blood disorders and are familiar with CAD. They can guide the diagnostic process and treat affected patients to maintain their red blood cells at optimal levels.
Is Cold agglutinin disease painful?
A common complaint among patients with cold agglutinin disease is painful fingers and toes with purplish discoloration associated with cold exposure.
Is cold agglutinin hereditary?
CAD is not a hereditary condition, and no genetic mutations are linked to CAD directly. However, a recent study identified mutations in two different genes in antibody-producing B-cells that were isolated from the bone marrow of 16 primary CAD patients ages 56 to 84.
How do people live with cold agglutinin disease?
Hygiene and avoiding infections Avoiding contact with sick people and avoiding crowded places. Eating well-cooked food and avoiding unhygienic eating places. Washing hands often. Brushing and flossing teeth regularly and getting regular dental care to avoid infections.
How many people in the world have cold agglutinin disease?
But depending on the study, looking at the prevalence, and it varies in various countries, about 5 to 20 per million people will have cold agglutinin disease, or CAD.
What happens when a patient with cold agglutinins body temperature falls?
Cold agglutinins are particular cold-reactive antibodies that react with red blood cells when the blood temperature drops below normal body temperature causing increased blood viscosity and red blood cell clumping.
Does cold agglutinin disease cause headaches?
Cold agglutinin disease manifests as acute or chronic hemolytic anemia, with associated pallor and fatigue. Symptoms during hemolytic ``crises'' may include severe pain in the back and legs, headache, vomiting, diarrhea, dark urine and hepatosplenomegaly.
What autoimmune disease makes you cold all the time?
The diseases most often linked with Raynaud's are autoimmune or connective tissue diseases such as: Lupus (systemic lupus erythematous) Scleroderma. CREST syndrome (a form of scleroderma)
What happens when a patient with cold agglutinin body temperature falls?
Cold agglutinins are particular cold-reactive antibodies that react with red blood cells when the blood temperature drops below normal body temperature causing increased blood viscosity and red blood cell clumping.
Can blood agglutination be cured?
If the causative antibodies are only active at room temperature, the agglutination can be reversed by heating the blood sample to 37 °C (99 °F). People with warm autoimmune hemolytic anemia may exhibit red cell agglutination that does not resolve on warming.
Is Cold agglutinin disease hereditary?
CAD is not a hereditary condition, and no genetic mutations are linked to CAD directly. However, a recent study identified mutations in two different genes in antibody-producing B-cells that were isolated from the bone marrow of 16 primary CAD patients ages 56 to 84.
What may be done to RBC before transfusion to a patient with cold agglutinin disease?
For people with CAD, it is vital to warm the blood to body temperature before the transfusion. If this in not done, the cold agglutinin antibodies that are already in the patient's blood may attack the red blood cells in the donor blood.
What is cold agglutinin disease?
Cold agglutinin disease is a rare type of autoimmune hemolytic anemia in which the body's immune system mistakenly attacks and destroys its own red blood cells. When affected people's blood is exposed to cold temperatures (32º to 50º F), certain proteins that normally attack bacteria (IgM antibodies) attach themselves to red blood cells and bind them together into clumps (agglutination). This eventually causes red blood cells to be prematurely destroyed (hemolysis) leading to anemia and other associated signs and symptoms. Cold agglutinin disease can be primary (unknown cause) or secondary, due to an underlying condition such as an infection, another autoimmune disease, or certain cancers. Treatment depends on many factors including the severity of the condition, the signs and symptoms present in each person, and the underlying cause. [1] [2] [3]
What age group is most affected by cold agglutinin?
Cold agglutinin disease most commonly affects adults who are of middle age and older. Some studies also report a slight bias in favor of females in the incidence of cold agglutinin disease, particularly in older populations.
How long does it take for cold agglutinin to go away?
For example, people with cold agglutinin disease caused by bacterial or viral infections tend to have an excellent prognosis; in these cases, the symptoms typically disappear within 6 months after the infection has resolved . Mild to moderate primary (unknown cause) cold agglutinin disease can also be associated with a good prognosis if excessive exposure to the cold is avoided. Those with cold agglutinin disease caused by HIV infection or certain types of cancer generally have a poor prognosis due to the nature of the underlying condition. [1]
What is the Coombs test?
An antiglobulin test (called the Coombs test) may be performed to determine the presence of a specific type of antibody. In people with cold agglutinin disease, the Coomb's test is almost always positive for immunoglobulin M (IgM).
Is cold agglutinin inherited?
Cold agglutinin disease is not an inherited condition. It is designated as either primary (unknown cause) or secondary (associated with or caused by another condition). In some cases, cold agglutinin may be multifactorial which means that multiple environmental factors and genes likely interact to predispose a person to developing the condition. [4] However, to our knowledge, no disease-causing genes have been identified and no familial cases have been reported.
Is cold agglutinin disease a primary or secondary condition?
Cold agglutinin disease is typically classified as primary (unknown cause) or secondary (caused by an underlying condition). Secondary cold agglutinin disease may be associated with: [1] [3]
Is cold agglutinin disease a good prognosis?
Mild to moderate primary ( unknown cause) cold agglutinin disease can also be associated with a good prognosis if excessive exposure to the cold is avoided. Those with cold agglut inin disease caused by HIV infection or certain types of cancer generally have a poor prognosis due to the nature of the underlying condition.
Why are cold agglutinins called cold agglutinins?
Some of these antibodies are called cold agglutinins because they’re triggered when your body’s temperature is below normal.
What is cold agglutinin disease?
Treatment. Taking Care of Yourself. Cold agglutinin disease (CAD) is a condition that makes your body’s immune system attack your red blood cells and destroy them. It’s triggered by cold temperatures, and it can cause problems that range from dizziness to heart failure. It’s also called cold antibody hemolytic anemia.
What is the blood test for CAD?
This is called a direct antiglobulin test or a Coombs test.
What causes cold agglutinin?
Doctors call that the primary, or idiopathic, form of the condition, and they don’t know why it happens. In other cases, another illness can bring on cold agglutinin disease. One of the most common conditions that triggers it is lymphoma, especially in people over 60. That's a type of cancer that affects your lymph nodes.
Can cold agglutinin cause a period?
In women, cold agglutinin disease sometimes keeps them from having periods. Some people who have cold agglutinin disease also may get a cold, numb feeling and loss of color in their fingers or toes, known as Raynaud’s phenomenon. Symptoms are typically worse in the winter, when temperature are lower.
What is the titer of a cold agglutinin test?
The result of a cold agglutinin test is typically reported as a titer, such as 1:64 or 1:512. A higher number means that there are more autoantibodies present.
What is cold agglutinin?
When the presence of cold agglutinins in a person's blood leads to significant RBC destruction, it can cause hemolytic anemia and lead to a low RBC count and hemoglobin. This rare form of autoimmune hemolytic anemia is known as cold agglutinin disease.
What are the higher titers of autoantibodies and those that react at warmer temperatures?
Higher titers of autoantibodies and those that react at warmer temperatures are associated with hemolytic anemia and worse symptoms.
Is cold agglutinin a secondary disease?
Cold agglutinin disease may be primary or secondary, induced by some other disease or condition such as: Mycoplasma pneumoniae infections —up to 75% of those affected will have increased cold agglutinins. Mono ( infectious mononucleosis )—more than 60% of those affected will have increased cold agglutinins, but anemia is rare with this infection.
What are the symptoms of a cold?
Symptoms may include: Fatigue, weakness, lack of energy, pale skin (pallor), dizziness and/or headaches from anemia. In some cases, painful bluish fingers, toes, ears, and the tip of the nose that occur with exposure to cold temperatures.
What is CBC test?
It may be used as a follow-up test after a complete blood count (C BC) shows a decrease in a person's red blood cell (RBC) count and hemoglobin, especially if these findings are linked to an exposure to cold temperatures.
Can cold agglutinin cause hemolytic anemia?
Secondary cold agglutinin disease may affect anyone and may be acute or chronic, temporary or persistent. It may cause hemolytic anemia to a greater or lesser degree and is associated with a variety of conditions. For details see the "What does the test result mean?" section in Common Questions below.
