Author: Mary Hulihan, DrPH
Hello. I am Dr Mary Hulihan, a health scientist in the Division of Blood Disorders at the Centers for Disease Control and Prevention. Today I would like to talk about the indications for blood transfusion in patients with sickle cell disease, the complications associated with these transfusions, and how you can help reduce the risk for these complications in your patients.
For many patients with sickle cell disease, red blood cell transfusions are an important part of medical care, reducing both mortality and morbidity. Both the National Heart, Lung, and Blood Institute[1] and the British Society for Haematology[2] have published documents that underscore the many unique aspects of care that must be taken into account when transfusing this patient population.
Although transfusions can be highly beneficial in treating sickle cell disease–related issues, they are not without risks. There are three scenarios when blood transfusions should be considered for patients with sickle cell disease. First, certain acute illnesses, such as acute chest syndrome or severe exacerbation of anemia with splenic or hepatic sequestration, may call for a simple blood transfusion. Other, more severe acute complications, such as recurring episodes of acute chest syndrome, multi–organ failure syndrome, or stroke, may require a more aggressive method, such as exchange transfusion.
Second, chronic transfusion is recommended for patients at high risk for stroke due to a history of stroke or as suggested by the results of transcranial Doppler screening.
Finally, simple transfusion is also recommended prior to elective surgery to reduce the high risk for postoperative sickle cell complications. It is equally important to recognize that most acute illnesses, especially pain crises, are not indications for transfusion.
When contemplating transfusion for patients with sickle cell disease, you should also weigh the potential complications of the procedure.
The most important are alloimmunization to minor red blood cell antigens and iron overload. Cardiovascular complications and hyperviscosity should also be considered, but these can be minimized by monitoring and careful management. Providers should reassure their patients that transmission of infection is very rare in the United States due to the use of modern and thorough approaches for screening donor blood.
There are ways to reduce or prevent these complications. First and foremost, transfusions should only be given when appropriate. When transfusions are indicated, a thorough medical history should be obtained, because patients often receive transfusions at more than one location.
Also, many centers recommend extended matching of all patients with sickle cell disease on at least C, E, and Kell antigens, and possibly more, depending on medical history and antibody screening results. Iron levels must also be monitored and a plan developed to lower these levels when excess iron is detected.
Nonmedical strategies also have been shown to reduce the rate of transfusion-related complications.
Donor recruitment targeted to African Americans is a way to minimize the differences between donor and recipient minor red cell antigens. Educating patients and their families on transfusion indications and complications allows for self-empowerment and advocacy.
Reliable interinstitutional blood bank communication is paramount for both reducing the risk of failing to detect transient antibodies and re-exposing the patient to an evanesced antibody with future transfusions.
Healthcare providers should recognize the appropriate indications for transfusions, the times when transfusion is not indicated, and steps for reducing and managing potential transfusion complications.
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