Cardiopulmonary bypass in a patient with cold agglutinin disease

Intravascular hemolysis is the greatest concern, as it can lead to severe anemia and release of free heme into the circulation. RBC agglutination in the vasculature or the CPB circuit can also occur. Data to guide therapy are very limited, and involvement of the consulting expert with expertise in cold agglutinin disease is advised.

Keeping the patient warm reduces hemolysis and RBC agglutination.
Plasmapheresis removes the cold agglutinating antibodies and lessens hemolysis and RBC agglutination. It is generally reserved for patients who cannot avoid hypothermia. If plasmapheresis is used, it should be done before the complement-blocking monoclonal antibody (eg, sutimlimab, eculizumab), since plasmapheresis removes antibodies including therapeutic monoclonal antibodies.
Complement blockade – only one agent is used. Sutimlimab is preferred because it is expected to provide more complete complement blockade. Complement blockade stops hemolysis but does not decrease RBC agglutination. If surgery is too urgent to give the dose preoperatively, it can be given during the procedure or postoperatively.
- Sutimlimab is a monoclonal antibody against complement component C1 that reduces hemolysis by the alternative and classical pathways. Only one dose is needed.
- Eculizumab is a monoclonal antibody against complement component C5 that reduces intravascular hemolysis mediated by the alternative pathway of complement. Repeat dosing may be appropriate if needed.

CPB: cardiopulmonary bypass; RBC: red blood cell.

* Refer to UpToDate for a discussion of therapies directed at the underlying cause, which may include infection, connective tissue disorder, lymphoproliferative disorder, or primary cold agglutinin disease-associated lymphoproliferative disorder.

¶ In most cases, hypothermia can be avoided. Exceptions include selected cases such as ascending aortic procedures. For all other procedures with CPB, surgery can be performed under normothermic conditions and cardioplegia techniques can be modified to keep the induction dose at a temperature above the thermal amplitude of the cold agglutinin, or the coronary arteries can be flushed with warm crystalloid. Other modifications are discussed in UpToDate.

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Cardiopulmonary bypass in a patient with cold agglutinin disease