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

Published online by Cambridge University Press:  21 May 2015

Andrew L. Merritt
Affiliation:
Department of Emergency Medicine, Keck/USC School of Medicine, Los Angeles, Calif.
Christopher Haiman
Affiliation:
Department of Preventive Medicine, Keck/USC School of Medicine, Los Angeles, Calif.
Sean O. Henderson*
Affiliation:
Department of Emergency Medicine, Keck/USC School of Medicine, Los Angeles, Calif. Department of Preventive Medicine, Keck/USC School of Medicine, Los Angeles, Calif.
*
Department of Emergency Medicine, LAC + USC Medical Center, 1200 N State St., Rm. 1011, Los Angeles CA 90033; 213 226-6667, fax 213 226-8044

Abstract

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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 suggests this may not be an effective therapy and may have significant neurologic sequelae. This paper reviews and summarizes the effectiveness and risks of blood transfusion 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 therapy 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.

Type
Education • Éducation
Copyright
Copyright © Canadian Association of Emergency Physicians 2006

References

1.Diggs, LW, Ching, RE. Pathology of sickle cell anaemia. South Med J 1934;27:839–45.Google Scholar
2.Bruno, D, Wigfall, DR, Zimmerman, SA, et al. Genitourinary complications of sickle cell disease. J Urol 2001;166:803–11.Google Scholar
3.Mantadakis, E, Cavender, JD, Rogers, ZR, et al. Prevalence of priapism in children and adolescents with sickle cell anemia. J Pediatr Hematol Oncol 1999;21:518–22.Google Scholar
4.McCollough, M, Sharieff, G. Renal and genitourinary tract disorders. In: Marx, J, Hockberger, R, Walls, R, editors. Rosen’s Emergency medicine: concepts and clinical practice. 6th ed. St. Louis (MO): Mosby; 2006. p. 2635–56.Google Scholar
5.Maan, Z, Arya, M, Patel, HR. Priapism — a review of medical management. Expert Opin Pharmacother 2003;4:2271–7.Google Scholar
6.Al Jam’a, AH. Al Dabbous, IA. Hydroxyurea in the treatment of sickle cell associated priapism. J Urol 1998;159:1642.CrossRefGoogle ScholarPubMed
7.El Mauhoub, M, El Bargathy, S, Sabharwal, HS, et al. Priapism in sickle cell anaemia: a case report. Ann Trop Paediatr 1991;11:371–2.CrossRefGoogle ScholarPubMed
8.Enwerem, EO, Endeley, EML, Holcombe, C, et al. Priapism in children with sickle cell disease. Indian Pediatr 1992;29:1048–9.Google Scholar
9.Fich, A, Rachmilewitz, EA. Priapism in a non-black with sickle cell anemia associated with α-thalassemia. Am J Hematol 1981;10:313–5.Google Scholar
10.Grace, DA, Winter, CC. Priapism: an appraisal of management of twenty-three patients. J Urol 1968;99:301–10.CrossRefGoogle ScholarPubMed
11.Gradisek, RE. Priapism in sickle cell disease. Ann Emerg Med 1983;12:510–2.CrossRefGoogle ScholarPubMed
12.Hasen, HB, Raines, SL. Priapism associated with sickle cell disease. J Urol 1962;88:71–6.Google Scholar
13.Karayalcin, G, Imran, M, Rosner, F. Priapism in sickle cell disease: report of five cases. Am J Med Sci 1972;264:289–93.CrossRefGoogle ScholarPubMed
14.Kinney, TR, Harris, MB, Russell, MO, et al. Priapism in association with sickle hemoglobinopathies in children. J Pediatr 1975;86:241–2.Google Scholar
15.Maconochie, IK, Scopes, JW. Priapism in a 6½-year-old boy with sickle cell disease. J R Soc Med 1988;81:606.Google Scholar
16.Seeler, RA. Intensive transfusion therapy for priapism in boys with sickle cell anemia. J Urol 1973;110:360–3.CrossRefGoogle ScholarPubMed
17.Seeler, RA. Priapism in children with sickle cell anemia. Clin Pediatr (Phila) 1971;10:418–9.CrossRefGoogle ScholarPubMed
18.Sousa, CM, Catoe, BL, Scott, RB. Studies in sickle cell anemia: priapism as a complication in children. J Pediatr 1962;60:52–4.CrossRefGoogle ScholarPubMed
19.Walker, EM, Mitchum, EN, Rous, SN, et al. Automated erythrocytopheresis for relief of priapism in sickle cell hemoglobinopathies. J Urol 1983;130:912–6.Google Scholar
20.Wasi, P, Na-Nakorn, S, Pootrakul, P, et al. A syndrome of hypertension, convulsion, and cerebral haemorrhage in thalassaemic patients after multiple blood-transfusions. Lancet 1978;2:602–4.Google Scholar
21.Royal, JE, Seeler, RA. Hypertension, convulsions, and cerebral hemorrhage in sickle-cell anemia patients after blood-transfusions. Lancet 1978;2:1207.Google Scholar
22.Rackoff, WR, Frempong, KO, Month, S, et al. Neurologic events after partial exchange transfusion for priapism in sickle cell disease. J Pediatr 1992;120:882–5.CrossRefGoogle ScholarPubMed
23.Miller, ST, Rao, SP, Dunn, EK, et al. Priapism in children with sickle cell disease. J Urol 1995;154:844–7.Google Scholar
24.Siegel, JF, Rich, MA, Brock, WA. Association of sickle cell disease, priapism, exchange transfusion and neurological events: ASPEN syndrome. J Urol 1993;150:1480–2.Google Scholar