Transfusion Dependence in Adult Sickle Cell Anemia

Transfusion is 1 of 2 disease-modifying therapies for adults with sickle cell anemia, the other being hydroxyurea, explains Morey Blinder, MD. There are several indications for transfusion in patients with sickle cell disease, with the best studied being prevention and management of stroke. Other defined indications for transfusion in patients with sickle cell include as a life-saving maneuver in acute chest syndrome, for multi-organ failure, in aplastic crisis, for hypersplenism, and as perioperative management.

Long-term benefits of transfusion in adult patients with sickle-cell include decreasing pain and allowing patients to lead a mainstream lifestyle. Whether or not transfusions can be stopped is a commonly asked question. There are certain situations when this is possible, says Blinder, including in a patient switching to hydroxyurea therapy, or for those who can achieve better pain management. In addition, stem cell transplantation is an emerging concept in this setting.

For patients with ongoing transfusion needs, Blinder initiates active therapy for iron overload when the serum ferritin level reaches >1000 μg/L. For those patients who have a lifelong history of transfusions but may not be on an ongoing transfusion program, the next step is to order magnetic resonance imaging to measure liver iron concentration and determine the need for chelation therapy. In these patients, Blinder administers chelation therapy when the iron level >1000 μg/L to 1500 μg/L.