The American Journal of Medicine
Volume 122, Issue 6 , Pages 507-512, June 2009

Complications Associated with Sickle Cell Trait: A Brief Narrative Review

  • Geoffrey Tsaras, MD, MPH

      Affiliations

    • Combined Medicine-Pediatrics, Bridgeport Hospital, Yale New Haven Health, Bridgeport, Connecticut
  • ,
  • Amma Owusu-Ansah, MD

      Affiliations

    • Staywell Health Center, Waterbury, Connecticut
  • ,
  • Freda Owusua Boateng, MD

      Affiliations

    • Combined Medicine-Pediatrics, Bridgeport Hospital, Yale New Haven Health, Bridgeport, Connecticut
  • ,
  • Yaw Amoateng-Adjepong, MD, MPH, PhD

      Affiliations

    • Combined Medicine-Pediatrics, Bridgeport Hospital, Yale New Haven Health, Bridgeport, Connecticut
    • Yale University School of Medicine, New Haven, Connecticut
    • Corresponding Author InformationRequests for reprints should be addressed to Yaw Amoateng-Adjepong, MD, MPH, PhD, Bridgeport Hospital, Yale New Haven Health, 267 Grant St, Bridgeport, CT 06610

published online 27 April 2009.

Abstract 

Sickle cell trait occurs in approximately 300 million people worldwide, with the highest prevalence of approximately 30% to 40% in sub-Saharan Africa. Long considered a benign carrier state with relative protection against severe malaria, sickle cell trait occasionally can be associated with significant morbidity and mortality. Sickle cell trait is exclusively associated with rare but often fatal renal medullary cancer. Current cumulative evidence is convincing for associations with hematuria, renal papillary necrosis, hyposthenuria, splenic infarction, exertional rhabdomyolysis, and exercise-related sudden death. Sickle cell trait is probably associated with complicated hyphema, venous thromboembolic events, fetal loss, neonatal deaths, and preeclampsia, and possibly associated with acute chest syndrome, asymptomatic bacteriuria, and anemia in pregnancy. There is insufficient evidence to suggest an independent association with retinopathy, cholelithiasis, priapism, leg ulcers, liver necrosis, avascular necrosis of the femoral head, and stroke. Despite these associations, the average life span of individuals with sickle cell trait is similar to that of the general population. Nonetheless, given the large number of people with sickle cell trait, it is important that physicians be aware of these associations.

Keywords: Complications, Hematuria, Renal medullary carcinoma, Sickle cell trait, Sudden death

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 Funding: None.

 Conflict of Interest: None.

 Authorship: All authors had access to the data and played a role in writing.

PII: S0002-9343(09)00115-6

doi:10.1016/j.amjmed.2008.12.020

The American Journal of Medicine
Volume 122, Issue 6 , Pages 507-512, June 2009