Onderaan dit artikel informatie op de Engelstalige website van C.A.D.
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Secondary cold agglutinin disease associated with Hashimoto disease
Abstract:
Our case involves a 53 year old woman.
Three years ago, she was investigated because of normal hemoglobin levels despite very a low erythrocyte count, which was revealed during the preoperative evaluation for ovarian cyst operation.
The Direct Coombs test was found to be positive against complement and negative against IgG.
Cold agglutinin titer was 1/448 (+).
Due to the polyclonal IgM increase, secondary cold agglutinin disease (CAD) was considered but no factor could be found that would lead to cold agglutinin disease.
During the post-operative follow-up, cold agglutinin titers increased with fluctuations in the patient.
Twenty-four months after transabdominal hysterectomy and bilateral salpingooopherectomy operation, diagnosis of Hashimoto disease was made upon detection of subclinical hypothyroidism.
No case of Hashimoto disease associated with CAD caused by polyclonal IgM has been reported until the present time.
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Wat informatie - Engelstalig - over C.A.D.:
Cold Agglutinin Disease can be caused by an underlying infectious disease (mycoplasma pneumonia, mumps, mononucleosis), lymphoproliferative disease (lymphoma, chronic lymphocytic leukemia), or connective tissue disorder (lupus).
This form, accounting for about 50% of CAD patients, is referred to as Secondary CAD, and tends to resolve if there is improvement in the underlying condition.
According to Norway's Drs. Sigbjørn Berentsen, Klaus Beiske, and Geir Tjonnfjørd in their most recent papers (October 2007 - see Links page), the other 50% of CAD patients are said to have Primary CAD.
It is now believed that 10% of those with Primary CAD are idiopathic (no known cause) and 90% are considered to have a low grade lymphoproliferative bone marrow disorder.
They find that "Except in the uncommon event of transformation, the lymphoproliferative B-cell disorders seldom, if ever, show features of a clinically overt lymphoma (3-4% of patients) even after decades."
Both Primary and Secondary Cold Agglutinin Disease are acquired conditions, not inherited.
One can have “warm agglutinins” and both warm and cold agglutinins at the same time.
Warm antibody disease is easier to treat.
Exposure to cold may significantly worsen anemia.
Many people with CAD also can be affected by two conditions called Raynaud’s phenomenon and acrocyanosis.
Raynaud’s phenomenon is triggered when cold constricts small arteries usually in the fingers and toes but can also affect other parts of the body such as the nose, ears, and face.
The digits become pale or bluish and can be numb, tingle, or have a burning sensation.
As re-warming takes place, the skin may be reddish then return to normal. (See Links Page)
Acrocyanosis is a painless disorder caused by constriction or spasms of small blood vessels due to cold.
People with the disease tend to be uncomfortable with sometimes swollen, sweaty, cold, bluish colored fingers, hands and feet. Warming decreases the bluishness.
Young people can develop CAD as a result of some other disease. It is generally self-limiting.
The onset of CAD can occur suddenly or gradually and typically first appears in individuals in their 50’s, 60’s or 70’s.
Some studies suggest that it affects slightly more women than men and this may be due to the fact that women comprise a greater proportion of the senior population.
Some signs of anemia are fatigue, headache, lack of energy and stamina, a yellow pallor and perhaps a yellowing of the eyes, dark urine, irritability, mental confusion, and an enlarged spleen.
Some individuals with CAD have low levels of cold antibodies and may not have overt symptoms while others having a very high antibody titer and low hemoglobin, may require blood transfusions as a temporary measure. (See Treatment Options Page)
This was forwarded by a CADdy and its origin is unkown:
Certain disease states are marked by the appearance of antibodies capable of agglutinating red blood cells at cold temperatures.
These cold agglutinins are usually IgM class autoantibodies which recognize specific sites on red blood cells.
They can be transiently associated with infections such as mycoplasma pneumonia and infectious mononucleosis, or be persistently present in diseases such as Waldenstrom’s macroglobulinemia, chronic lymphocytic leukemias, and idiopathic cold agglutinin disease.
Post-infectious cold agglutinins are usually polyclonal, whereas cold agglutinins associated with B-cell neoplasms are generally monoclonal. The effects of cold agglutinins can range from minimal to severe hemolytic anemia.
The degree of red blood cell destruction depends upon complex factors, including the titer of the antibody (greatest serum dilution able to agglutinate red cells) and the thermal amplitude (maximum temperature at which agglutination of red cells still occurs.)
Generally, high titers occur with high thermal amplitudes.
However, there are cases in which even low titers can be clinically significant if the reactive temperature is very high.
If a cold agglutinin has a high thermal amplitude, special measures must be taken to keep the patient’s body temperature above the reaction temperature.
In such cases, ordinary room temperature may trigger agglutination.
Blood transfusions pose special problems unless the cross matching has been done at 37°C. and the transfused cells have been washed to minimize the amount of complement in the transfusion.
When one is diagnosed with Cold Agglutinins, in addition to a CBC (complete blood count), doctors customarily do a battery of tests to determine what is causing the Autoimmune Hemolytic Anemia.
Often, a bone marrow biopsy is performed to rule out any underlying lymphoproliferative disease such as lymphoma or leukemia.
Wat overige informatie:
The following are some suggestions we offer from our own personal experiences. If you have anything to add to our list, please contact us.
When you have CAD, you need to be certain that you keep warm at all times. For some, anything cold entering your body could cause an autoimmune response resulting in red cell destruction.
Dressing appropriately is the first line of defense:
Zie meer over adviezen via deze link:
http://www.coldagglutinindisease.org/ma ... ingcad.htm
Link naar de website van C.A.D.:
http://www.coldagglutinindisease.org/index.html
