Is cold agglutinin disease life threatening?
Is cold agglutinin disease life threatening?
For patients with primary CAD (or CAD of unknown cause), life expectancy is usually normal if efforts are made to limit exposure to cold. However, crises caused by cold can be very damaging and, for some patients, lead to problems like those of the heart that impact life expectancy.
How is cold agglutinin disease diagnosed?
In most cases, the diagnosis is based on evidence of hemolytic anemia (from symptoms and/or blood tests). A person may also be physically examined for spleen or liver enlargement. An antiglobulin test (called the Coombs test) may be performed to determine the presence of a specific type of antibody .
Is there a cure for cold agglutinin disease?
Cold agglutinin disease is a rare, acquired chronic autoimmune hemolytic condition that destroys red blood cells. It leads to chronic anemia, severe fatigue, and potentially fatal thrombotic events. No drug has yet been approved to treat cold agglutinin disease.
What triggers cold agglutinin?
Causes of polyclonal secondary cold agglutinin disease include the following: Mycoplasma infections – M pneumoniae. Viral infections – Infectious mononucleosis due to Epstein-Barr virus (EBV) or CMV. Viral infections, other – Mumps, varicella, rubella, adenovirus, HIV, influenza, hepatitis C.
How do people live with cold agglutinin disease?
Living With Cold Agglutinin Disease
- Wearing gloves or mittens when taking food out of the refrigerator or freezer.
- Wearing a hat, scarf, and a coat with snug cuffs during cold weather.
- Turning down air conditioning or dressing warmly while in an air-conditioned space.
- Warming up the car before driving in cold weather.
Why would a patient with a positive cold agglutinin test be tested?
A positive titer may mean that the person tested has cold agglutinin 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.
Is cold agglutinin disease an autoimmune disease?
INTRODUCTION Cold agglutinin disease (CAD) is a form of autoimmune hemolytic anemia (AIHA) in which cold agglutinins (agglutinating autoantibodies with an optimum temperature of 3 to 4°C) can cause clinical symptoms related to agglutination of red blood cells in cooler parts of the body and hemolytic anemia.
What does it mean to have cold agglutinin disease?
Cold agglutinin disease is a rare disorder affecting 15% of patients with autoimmune hemolytic anemia. The disease is defined by the presence of cold agglutinins which cause red blood cells to clump together (agglutinate) at low temperatures.
When does cold agglutinin cause hemolytic anemia?
Cold agglutinins cause red blood cells to clump together (agglutinate) at low temperatures. Cold agglutinin disease (CAD) occurs more frequently after the age of 55. Clinical features: Cold agglutinin disease (CAD) manifests as acute or chronic hemolytic anemia, with associated pallor and fatigue.
How does the complement pathway work in cold agglutinin disease?
In cold agglutinin disease, certain abnormal bone marrow cells produce antibodies called cold agglutinins, which activate a part of the immune system known as the complement pathway. This activation results in the constant destruction of red blood cells, known as hemolysis. Here’s how it works:
How is rituximab used to treat cold agglutinin?
Rituximab is an intravenous (IV) medication that helps to destroy the white blood cells that produce the antibodies against red blood cells, called B-cells. The hope is that when your B-cells regenerate, they will no longer make the antibody. This is typically used for patients with severe disease.
What is the prognosis of cold agglutinin disease?
The outlook for people with secondary cold agglutinin disease caused by bacterial or viral infections is excellent prognosis and cold agglutinin disease symptoms usually go away within 6 months after the infection has resolved.
How do I manage cold agglutinin disease?
Cold Agglutinin Disease Treatment & Management Approach Considerations. The treatment of cold agglutinin disease depends on the gravity of the clinical manifestations, as determined by the characteristics of the antibody and the presence of associated disease Chemotherapy and Immunosuppression. Transfusions. Management of Surgical Patients. Transfer. Deterrence. Long-Term Monitoring.
What are rare causes of cold agglutinin disease?
Cold agglutinin disease (CAD) is a rare type of autoimmune hemolytic anemia-an anemia that develops when your immune system attacks and destroys your red blood cells. The most common cause of AIHA is due to a warm autoantibody .
What causes a cold agglutinin?
Cold agglutinin disease is a form of autoimmune hemolytic anemia caused by cold-reacting autoantibodies (a type of protein produced by the immune system). Primary cold agglutinin disease is usually associated with monoclonal (produced from a single ancestral cell by repeated cellular replication) cold-reacting autoantibodies.