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Cold Agglutinin Disease: Treatment Considerations


An overview of the roles of plasmapheresis, IVIG, transfusions, and corticosteroids as treatment for patients with cold agglutinin disease.

Neil Minkoff, MD: Let me push a little more on you, not negatively but to get to the next level of detail. Some of the things you mentioned were plasma exchange, transfusions, and so on. How do you decide which patients get plasmapheresis vs IVIG [intravenous immunoglobulin] and who gets a transfusion? Can you help us? Can you give us a little more brass tacks on that?

Mihir Raval, MD, MPH: Definitely. When you diagnose a patient, the major thing that you’d be treating is their symptoms from anemia. Transfusion, when and where needed, isn’t contraindicated. Unlike some other autoimmune hemolytic anemia, in cold agglutinin disease [CAD] or cold agglutinin anemia, you don’t have to worry about giving them an unmatched or noncompatible blood product. If the patient does require transfusion, go ahead and order that. Simultaneously, remember to warm the blood product. That’s 1 thing I always do: If the patient needs transfusion, give it to them.

Plasma exchange is something I reserve for preprocedure. If I have somebody going for a bypass surgery with cold agglutinin disease, I don’t want them to have a cold cardioplegia, which means when they do the bypass, they lower the temperature significantly. You want to do plasma exchange within 1 to 2 days before the surgery to lower their antibody counts so they don’t trigger that. Plus, try to see if anesthesia and cardiothoracic can do warm cardioplegia for those patients. IVIG doesn’t really have strong data in treating cold agglutinin disease, so we don’t use IVIG. But cold avoidance, transfusion whenever it’s needed, avoiding atmospheres inside the hospitals, warming the blood product, making sure that you use plasma exchange preprocedure if needed if they have high titer, using a warm room for the plasma exchange, and also making sure patients aren’t using cooling blankets when they need to or warm cardioplegia when they need to under surgery—those are some of the things you would commonly do for patients even before you talk about chemotherapy, chemotherapy-immunotherapy, or a similar complement-directed treatment.

Neil Minkoff, MD: I want to get into that next, but I’m going to ask you 1 more question before we make that turn, which is about the role of corticosteroids. Concerning the role of corticosteroids, inpatient and outpatient, what are the times that you find that it’s useful and warranted?

Mihir Raval, MD, MPH: Typically, warm autoimmune hemolytic anemia is where you would use the corticosteroids. The only time, for inpatients with CAD, that corticosteroids might be useful is, 1, if you have a mixed hemolytic anemic—if you have an IgG and IgM, or if you feel that thermal amplitude isn’t 3°C or 4°C but is higher. If they have symptoms of hemolytic anemia at close to normal room temperature, there are some data using steroids in that situation as well. Otherwise, there’s no role for steroids, unlike the warm autoimmune hemolytic anemia.

Transcript edited for clarity.

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