Myth: Blood transfusion is effective for sickle cell anemia-associated priapism

Abstract

Objective: Priapism is a recognized complication of sickle cell anemia (SCA). When initial conventional treatments fail, simple or exchange blood transfusion has been advocated as a secondary intervention. However, recent literature suggest this may not be an effective therapy and may have significant neurologic sequelae. This paper reviews and summarizes the effectiveness and risk of blood tranfusion compared with conventional priapism therapy. Methods: All relevant papers identified from a MEDLINE search were systematically examined for data related to the use of blood transfusion in the setting of priapism due to SCA. The effectiveness of conventional therapy was compared with transfusion therapy using the outcome of "time to detumescence" (TTD). In addition, papers documenting adverse neurologic sequela were reviewed and summarized. Results: Forty-two case reports were identified containing complete information with regard to patient age and TTD. The mean TTD was 8.0 days with conventional therapy (n = 16) and 10.8 days with blood transfusion therapy (n=26). Adverse neurologic sequelae from blood transfusion theraphy was described in 9 cases, with long term outcomes ranging from complete resolution to severe residual deficits. Conclusion: The current literature does not support the contention that blood transfusion is an effective therapy in the treatment of priapism due to SCA, as defined by an acceleration of TTD. In fact, numerous reports suggest that serious neurologic sequelae may result from this treatment. We feel the routine use of this therapy cannot be recommended.