Available Treatments for Cold Agglutinin Disease

Rituximab

Rituximab is used as a first-line treatment for CAD and is effective as a monotherapy in about half of all cases. It is an antibody that binds to the immune cells that produce autoantibodies. With fewer autoantibodies around to bind to red blood cells, there’s less clumping of these blood cells and fewer immune attacks.

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Rituximab-Fludarabine

Rituximab-fludarabine combination therapy has been recommended as a first-line treatment for CAD. Rituximab works by reducing the number of autoantibodies, while fludarabine inhibits DNA synthesis. Blocking DNA synthesis strongly affects those cells that rely on rapid cell division — like immune cells.

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Erythropoietin

Erythropoietin (EPO) is a hormone produced by the kidneys that helps in the production of red blood cells. The administration of EPO could help the body produce more red blood cells to compensate for their loss in severe forms of CAD.  Excessive use of EPO, however, can lead to conditions associated with a rapid rise in hemoglobin concentrations, including cardiovascular events and blood clots.

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Blood Transfusions

A blood transfusion is usually used only in severe cases of CAD. It is a medical procedure in which healthy blood, or specific blood components, are given to patients through the vein. In CAD, the transfusion of washed red blood cells from compatible donors serves as a temporary but sometimes necessary treatment.

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Plasmapheresis

Plasmapheresis is a treatment that has been used in severe cases of CAD and for those who do not respond to other treatments. It is a process in which the plasma from a patient’s blood is replaced with fresh plasma or a plasma substitute, removing harmful components such as the autoantibodies. Removing autoantibodies may help reduce damage to red blood cells and alleviate symptoms.

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