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sickle cell trait; hemoglobin S trait

Carriers of a single hemoglobin S gene. Pathology: - renal papillary necrosis accounts for 1/2 of hematuria Clinical manifestations: 1) asymptomatic 2) painless hematuria Laboratory: 1) occasional hematuria 2) occasional hyposthenuria 3) hemoglobin electrophoresis: Hgb A 60%, Hgb S 40% 4) HgbA1c may underestimate average blood glucose by ~5% [7] Complications: - renal medullary carcinoma - increased risk of renal disease - chronic renal failure [4,9], including end-stage renal disease [9] - hematuria (RR=2) - albuminuria (RR=2) [8] - splenic rupture at high altitudes - venous thromboembolism [8] - sudden death during extreme conditions - exertional rhabdomyolysis - similar changes in cardiovascular fitness - no excess risk for hypertension, diabetes, or metabolic syndrome [6] - higher prevalence of type 2 diabetes, hypertension, retinal disorders [9] Management: - diagnostic workup should not differ from patients with normal hemoglobin

Interactions

disease interactions

General

hemoglobinopathy

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 11, 16, 17, 18. American College of Physicians, Philadelphia 1998, 2012, 2015, 2018.
  2. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 648
  3. Tsaras G, Owusu-Ansah A, Boateng FO, Amoateng-Adjepong Y. Complications associated with sickle cell trait: a brief narrative review. Am J Med. 2009 Jun;122(6):507-12 PMID: 19393983
  4. Naik RP et al. Association of sickle cell trait with chronic kidney disease and albuminuria in African Americans. JAMA 2014 Nov 13 PMID: 25393378
  5. Asplund CA, O'Connor FG. Challenging Return to Play Decisions: Heat Stroke, Exertional Rhabdomyolysis, and Exertional Collapse Associated With Sickle Cell Trait. Sports Health. 2016 Mar-Apr;8(2):117-25. PMID: 26896216 - Saxena P, Chavarria C, Thurlow J. Rhabdomyolysis in a Sickle Cell Trait Positive Active Duty Male Soldier. US Army Med Dep J. 2016 Jan-Mar:20-3. PMID: 26874092 - Nelson DA, Deuster PA, Carter R 3rd et al., Sickle Cell Trait, Rhabdomyolysis, and Mortality among U.S. Army Soldiers. N Engl J Med. 2016 Aug 4;375(5):435-42. PMID: 27518662
  6. Liem RI, Chan C, Vu TT et al. Longitudinal association among sickle cell trait, fitness, and cardiovascular disease risk factors in African Americans in CARDIA. Blood 2016 Nov 16 PMID: 27856464
  7. Lacy ME, Wellenius GA, Sumner AE et al Association of Sickle Cell Trait With Hemoglobin A1c in African Americans. JAMA. 2017;317(5):507-515 PMID: 28170479 http://jamanetwork.com/journals/jama/article-abstract/2600468 - Bleyer AJ, Aloi JA Sickle Cell Trait and Interpretation of Hemoglobin A1c Levels. JAMA. 2017;317(5):481-482 PMID: 28170462 http://jamanetwork.com/journals/jama/article-abstract/260044
  8. Naik RP, Smith-Whitley K, Hassell KL et al. Clinical outcomes associated with sickle cell trait: A systematic review. Ann Intern Med 2018 Oct 30; PMID: 30383109
  9. Hulsizer J et al. Sickle cell trait and risk for common diseases: Evidence from the UK Biobank. Am J Med 2022 Aug; 135:e279. PMID: 35461807 https://www.amjmed.com/article/S0002-9343(22)00282-0/fulltext