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Cold Agglutinin Disease

What is Cold Agglutinin Disease?

Cold Agglutinin Disease is an autoimmune disorder yeilding the high presence of circulating antibodies directed against red blood cells.


Cold Agglutinin Disease is an autoimmune disorder yeilding the high presence of circulating antibodies directed against red blood cells.
Acknowledgement of Cold Agglutinin Disease has not been added yet.
Prevalence Information of Cold Agglutinin Disease has not been added yet.
Synonyms for Cold Agglutinin Disease has not been added yet.
Cold agglutinin disease is not an inherited condition, it can be primary (idiopathic) or secondary, caused by an underlying condition ( Secondary cold agglutinin disease has been associated with: Mycoplasma, legionnaires' disease, syphilis, listeriosis, E. Coli, epstein-barr virus, cytomegalovirus, mumps, varicella, rubella, adenovirus, HIV,influenza, hepatitis C, malaria, trypanosomiasis, systemic lupus erythematosus, lymphoma, chronic lymphocytic leukemia, waldenström macroglobulinemia, multiple myeloma, and Kaposi sarcoma ( Secondary cold agglutinin disease is more common ( It can also be multifactorial, which means that multiple environmental factors and genes likely interact to predispose a person to developing the condition. However, to our knowledge, no disease-causing genes have been identified and no familial cases have been reported (
As the number or red blood cells drop, affected people typically experience anemia, which may be associated with pallor, weakness, fatigue, irritability, headaches, and/or dizziness ( Other signs and symptoms of cold agglutinin disease vary, but may include: Painful fingers and toes with purplish discoloration, abnormal behavior, amenorrhea, gastrointestinal issues, dark urine, severe pain in the back and legs enlargement of the spleen, jaundice, heart failure, shock, arthralgia, autoimmune hemolytic anemia, nausea/vomiting (,,
There are a wide variety of tests that can be taken to diagnose the disorder ( There’s also a long list of differential diagnoses: Acute anemia, cholangitis, chronic anemia, donath-landsteiner hemolytic anemia, evans syndrome, lymphoproliferative disorders, non-hodgkin lymphoma, systemic lupus erythematosus (SLE), cryoglobulinemia, warm antibody–mediated autoimmune hemolytic anemia (AIHA) - Some cases of cold agglutinin disease may be confused with this condition, particularly those in which the antibody has a high thermal amplitude, episodic hemoglobinuria following cold exposure, neoplasms - especially of the lymphoid type, drug-induced immune hemolytic anemia, rheumatologic diseases manifesting raynaud syndrome, heparin-induced thrombocytopenia/thrombosis syndrome - patients may have painful digits; anemia could be due to bleeding, but other manifestations are different, systemic vasculitis: sometimes with hemolysis (systemic lupus and scleroderma), erythromelalgia in association with primary thrombocythemia and painful fingers and toes, and malaria (
Diagnostic tests of Cold Agglutinin Disease has not been added yet
The treatment of the disease should be chosen based on the severity of the condition, the symptoms and the underlying cause ( In most cases, the disease is not very severe and can be managed just by wearing protective clothing. If it is a severe case there are several treatment options. Splenectomy: If the patient has splenomegaly, then the disease may respond to splenectomy. Diet and Activity: Patients with cold agglutinin disease should include good sources of folic acid, such as fresh fruits and vegetables, in their diet. Activities for these individuals should be less strenuous than those for healthy people, particularly for patients with anemia. Glucocorticoids: May occasionally work if an underlying warm antibody–induced hemolytic anemia exists. Rituximab:The anti-CD20 monoclonal antibody rituximab depletes B-lymphocytes, thereby interfering with the production of cold agglutinin. Plasmapheresis: Effectively, albeit temporarily, removes IgM antibody from plasma, reducing its concentration. This procedure is valuable for emergencies and allows time for drugs to have an effect (
Depending on the the severity, good long-term prognosis is predicted for patients with primary cold agglutinin disease ( Prognosis for patients with secondary cold agglutinin disease depends on the underlying cause. Cold agglutinin disease associated with HIV infection may have a relatively poor prognosis due to the nature of the underlying disease. The same applies to cases associated with lymphoma, with the prognosis dependent on remission of the underlying malignancy. Other general complications include: Brisk hemolysis due to cold exposure, ischemic complications at exposed sites due to prolonged cold exposure, other symptoms related to severe anemia, development of malignant disease during follow-up care of a patient with idiopathic chronic cold agglutinin disease, shock or congestive heart failure- resulting from severe hemolysis and anemia, and peripheral gangrene (
Tips or Suggestions of Cold Agglutinin Disease has not been added yet.
References of Cold Agglutinin Disease has not been added yet.
Experiences Created by heatheratsss
Last updated 10 Feb 2009, 02:05 PM

Posted by katherinepringle
10 Feb 2009, 02:05 PM

Hi Heather, I also suffer from CAD. I have been treated with prednisone also but more recently with Rituximab which is given by infusion. This has successfully kept my haemoglobin at a better level and more stable. It doesn't work for eveyone but definitely worth a try. You have 1 infusion every week for 4 weeks. Other advice is to really keep warm - don't drink cold drinks or food straight from fridge. Let me know if you want to know more. Best wishes, Katherine

Posted by heatheratsss
10 Feb 2009, 01:52 PM

My husband has been suffering from what his hematologist calls cold agglutin hemolytic anemia for at least 3 years (at first we thought it was repeated bouts of the flu). Does any one have any advice for slowing down recurrences? Any treatment options besides prednisone? Thanks -- Heather

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I have CLL with secondary CAD Last year I had 6 months of FCR for 3days a month in St Vincent's Private Hospital located in Melbourne Victoria Australia
My mom is 81 years old and was diagnosed with this disease. Her mother died of autoimmune hemolytic anemia as well but this isn't supposed to be hereditary. My mom received 6 blood transfusions...
My husband has cold agglutin hemolytic anemia.
My dad John Harms, 89, has been diagnosed with hemolytic anemia and cold agglutinin disease about 20 years. He also has lymphoma which is in remission and bronchiectasis which is treated with Tobi...

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Created by heatheratsss | Last updated 10 Feb 2009, 02:05 PM


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