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The treatment of cold agglutinin disease depends on the gravity of the symptoms as determined by the characteristics of the antibody and the presence of associated disease.
Cold agglutinin disease may be managed successfully using protective measures (clothing) alone in most cases.
Special protective clothing is sometimes necessary in extreme cases. Therapy is directed at serious symptoms and the underlying disorder, if any is found.
The idiopathic variety of cold agglutinin disease is generally a benign disorder with prolonged survival and spontaneous exacerbations and remissions in the course of the disease.
Acute postinfectious syndromes usually resolve spontaneously.
Anemia is generally mild.
Only patients who have serious symptoms related to anemia or have a Raynaud-type syndrome that constitutes a threat to life or quality of life require active therapy.
The presence of an associated malignancy requires specific therapy.
Patients with cold agglutinin disease should include good sources of
folic acid, such as fresh fruits and vegetables, in their diet.
Chemotherapeutic agents should be used under appropriate circumstances, such as for an associated malignancy.
Rituximab interfer with the production of cold agglutinin.
Plasmapheresis is valuable for emergencies and allows time for drugs to have an effect.
Periodic checkups may vary from daily to weekly or monthly and may eventually occur as infrequently as every 2-3 months.
Make reevaluations more often in colder weather than in warmer weather.
Monitor blood cell counts and observe for infection, renal function, development of
lymphoma, and evidence of
ischemia.
The following tests can be performed monthly until the cold agglutinin disease has resolved:
CBC
Reticulocyte count
Urinalysis
Direct antiglobulin test
Cold agglutinin titer
Avoid unnecessary transfusions, because cold agglutinin disease is usually self-limited.
RBC transfusion is indicated in severe, acute disease.
The response to transfused RBCs may be transient, but it can result in significant improvement in an acutely ill patient.
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