Cold agglutinin disease
Other Names for this Disease
- Anemia, hemolytic, cold antibody
- Chronic cold agglutinin disease
- Cold agglutinin syndrome
What are the signs and symptoms of cold agglutinin disease?
What causes cold agglutinin disease?
Is cold agglutinin disease inherited?
How is cold agglutinin disease diagnosed?
How might cold agglutinin disease be treated?
The blood of patients with cold agglutinin disease, when exposed to cold temperatures, behaves in ways that are quite different from the blood of healthy people. In affected individuals, certain proteins that normally attack bacteria (IgM antibodies), attach themselves to red blood cells and bind them together into clumps (agglutination). The antibodies activate other components of the blood, eventually causing red blood cells to be prematurely destroyed (hemolysis), thus causing anemia.
Subsequent signs and symptoms can include weakness, dizziness, fatigue, headache, ringing in the ears (tinnitus), and spots before the eyes. Additional symptoms \may include irritability, bizarre behavior, absence of menstrual cycles in affected females (amenorrhea), gastrointestinal complaints, low levels of circulating red blood cells (anemia), enlargement of the spleen (splenomegaly), and/or persistent yellowing of the skin, mucous membranes, and whites of the eyes (jaundice). In some cases, affected individuals may experience sweating and coldness of the fingers and/or toes, and uneven bluish or reddish discoloration of the skin of the fingers, toes, ankles, and wrists (called acrocyanosis or Raynaud's sign). Heart failure or shock may result in some cases.
Detailed information about the various tests used to make a diagnosis of CAD is available on Medscape Reference's Web site.
When people do not respond to corticosteroids or when the corticosteroid causes intolerable side effects, surgery to remove the spleen (splenectomy) may be considered. If the destruction of red blood cells persists after removal of the spleen or when surgery cannot be performed, immunosuppressive drugs, such as cyclophosphamide or azathioprine, may be prescribed.
Plasmapheresis, which involves filtering blood to remove antibodies, is occasionally helpful when other treatments fail. When red blood cell destruction is severe, blood transfusions are sometimes needed, but they do not treat the cause of the anemia and provide only temporary relief. In cases where blood transfusions are necessary, certain guidelines must be followed because of the temperature sensitivities involved.
- Harper JL. Pediatric Cold agglutinin disease. eMedicine. August 2011; http://emedicine.medscape.com/article/954954-overview. Accessed 1/20/2012.
- Georgy S. Cold agglutinin disease. eMedicine. November 2010; http://emedicine.medscape.com/article/135327-overview. Accessed 1/20/2012.
- Marc Michel. Cold agglutinin disease. Orphanet. August, 2010; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=56425. Accessed 3/11/2014.
- Cold antibody hemolytic anemia. National Organization for Rare Disorders (NORD). 2006; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/723/viewAbstract. Accessed 1/20/2012.
- Marc Michel. Cold agglutinin disease. Orphanet. August, 2010; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=56425. Accessed 5/15/2014.
- Anemia, Hemolytic, Cold Antibody. NORD. March 27, 2008; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/723/viewAbstract. Accessed 3/11/2014.