Pre-treatments helped limit complications of CAD during surgery

86-year-old CAD patient received pre-treatment with rituximab and plasmapheresis

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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In people with cold agglutinin disease (CAD), pre-treatment with rituximab and plasma exchange may help limit complications during surgeries where the patient’s body temperature is decreased, a new report illustrates.

The case study, “Total arch replacement for an aortic arch aneurysm with cold agglutinin disease after rituximab and plasmapheresis,” was published in the Journal of Cardiothoracic Surgery.

CAD is an autoimmune disease characterized by the excessive production of self-reactive antibodies, called cold agglutinins, that wrongly bind to red blood cells at cold temperatures.

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This causes the cells to clump, or agglutinate, and be marked for destruction, limiting the body’s ability to transport oxygen throughout the body and causing symptoms such as fatigue.

CAD can be classified as primary when its cause is unknown and secondary when it occurs due to an underlying condition, generally an infection, another autoimmune disease, or cancer.

Because CAD symptoms worsen at low temperatures, patients are at a higher risk of complications during surgeries in the heart and major blood vessels that require hypothermic circulatory arrest — a procedure in which blood flow is stopped and the body is kept at low temperatures (hypothermia).

Pre-treatment with rituximab and plasmapheresis

Scientists in Japan now report the case of an 86-year-old man incidentally diagnosed with CAD who underwent an uneventful major blood vessel surgery after receiving pre-treatment with rituximab and plasmapheresis.

Rituximab, which works by killing antibody-producing B-cells, is commonly used off-label as a first-line therapy in CAD. Plasmapheresis, or plasma exchange, is a blood-cleaning procedure used in CAD to help reduce the levels of cold agglutinins.

The man sought medical attention due to chest pain and was diagnosed with a type A aortic dissection, which is a tear in the part of the aorta, the body’s main artery, where it exits the heart.

He also had a history of rheumatoid arthritis, an autoimmune disease that mainly affects the joints, and a thoracic aortic aneurysm, which is a bulge in the part of the aorta that runs through the chest.

The man was scheduled to undergo urgent surgery to address both aortic injuries, but pre-surgery lab tests revealed blood clotting and high levels of cold agglutinin, indicating the presence of CAD.

As the surgery is typically performed under moderate or deep hypothermia and circulatory arrest and his aorta-related condition was not getting substantially worse, the decision was made to address his CAD before moving to surgery.

“For cases such as ours, when there is time before surgery, it is advantageous to consult with hematologists to determine the optimal treatment for CAD,” the scientists wrote.

Rituximab started two months before surgery

Starting two months before the surgery, the man received weekly infusions of rituximab, at a dose of 375 mg/m2, weekly for four weeks. Then two days before the surgery, he also underwent plasma exchange.

Following these treatments, the patient underwent arch replacement surgery, which aims to replace the damaged section of the blood vessel with a human-made prosthetic.

In addition to CAD treatment before surgery, the researchers employed a few cautionary techniques during the surgery itself, aiming to minimize CAD complications. The procedure was done under mild, instead of moderate-to-deep, hypothermia, and the solution infused into the heart to stop it temporarily was kept warm to reduce the risk of cold-related problems.

Following surgery, which was conducted without complications, the man experienced bleeding, for which he received a blood transfusion. In the following weeks, he also showed higher-than-normal levels of markers of red blood cell destruction and low levels of certain immune cells that were subsequently resolved without any treatment.

He was discharged from the hospital about a month after surgery, without any signs of neurological problems or infection.

Surgery was ‘safely performed’

“After consulting with hematologists, and after rituximab therapy and plasmapheresis were performed before surgery, arch replacement surgery under mild hypothermia conditions with CAD was safely performed,” the researchers wrote.

However, “because CAD could be associated with rheumatoid arthritis, CAD remission did not occur,” the researchers wrote, adding that treatment for rheumatoid arthritis “may improve CAD; however, the response time may be longer.”

“To our knowledge, this is the first reported case of total arch replacement with CAD after rituximab and plasmapheresis. Therefore, further research is needed,” they concluded.