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

References 

  1. Ashley-Koch A, Yang Q, Olney RS. Sickle hemoglobin (HbS) allele and sickle cell disease: a HUGE Review. Am J Epidemiol. 2000;151:839–845
  2. Powars DR, Meiselman HJ, Fisher TC, et al. Beta-S gene cluster haplotypes modulate hematologic and hemorheologic expression in sickle cell anemia (Use in predicting clinical severity). Am J Pediatr Hematol Oncol. 1994;16:55–61
  3. World Health Organization. Sickle cell anemia. Report by the secretariat. 59th World Health Assembly. April 2 2006;
  4. Salamah MM, Mallouh AA, Hamdan JA. Acute splenic sequestration crisis in Saudi children with sickle cell disease. Ann Trop Paediatr. 1989;9:115–117
  5. Lorey FW, Arnopp J, Cunningham GC. Distribution of hemoglobinopathy variants by ethnicity in a multiethnic state. Genet Epidemiol. 1996;13:501–512
  6. Steinberg MH, Embury SH. α-thalassemia in blacks: genetic and clinical aspects and interactions with the sickle hemoglobin gene. Blood. 1986;68:985–990
  7. Lange RD, Minnich V, Moore CV. In vitro differences in behavior of sickle cell anemia and trait erythrocytes produced by variations in O2 tension and pH. J Lab Clin Med. 1950;36:848
  8. Kark JA, Ward FT. Exercise and hemoglobin S. Semin Hematol. 1994;31:181–225
  9. Nance WE, Grore J. Genetic determinations of phenotypic variation in sickle cell trait. Science. 1972;177:716–718
  10. Diggs LW, Ahmann CF, Bibb J. Incidence and significance of sickle cell trait. Ann Intern Med. 1933;7:769–778
  11. Sears DA. The morbidity of sickle cell trait: a review of the literature. Am J Med. 1978;64:1021–1036
  12. Ashcroft MT, Desai P. Mortality and morbidity in Jamaican adults with sickle cell trait and with normal hemoglobin followed up for 12 years. Lancet. 1976;2:784
  13. Castro O, Scott RB. Red blood cell counts and indices in sickle cell trait in a black American population. Hemoglobin. 1985;9:65–67
  14. Ould Amar AK. Red blood cells from donors with sickle cell trait: a safety issue for transfusion?. Transfus Med. 2006;16:248–253
  15. American Red Cross. Blood donation eligibility guidelines. http://www.redcross.org/services/biomed/0,1082,0_557_,00.html
  16. Ould Amar AK, Kerob-Bauchet B, Robert P, et al. Assessment of qualitative functional parameters of stored red blood cells from donors with sickle cell trait (AS) or with heterozygotes AC status. Transfus Clin Biol. 1996;3:225–233
  17. Davis CJ, Mostofi FK, Sestarhenn IA. Renal medullary carcinoma (The seventh sickle cell nephropathy). Am J Surg Pathol. 1995;19:1–11
  18. Watanabe IC, Billis A, Guimares MS, et al. Renal medullary carcinoma: report of seven cases from Brazil. Mod Pathol. 2007;20:914–920
  19. Noguera-Irizarry WG, Hibshoosh H, Papadopoulos KP. Renal medullary carcinoma: case report and review of the literature. Am J Clin Oncol. 2003;26:489–492
  20. Hellar P, Best WR, Nelson RB, Beckel J. Clinical implications of sickle cell trait and glucose -6-phosphate dehydrogenase deficiency in hospitalized black male patients. N Engl J Med. 1979;300:1001–1005
  21. Kiryluk K, Jadron A, Gupta M, Radhakrishan J. Sickle cell trait and gross hematuria. Kidney Int. 2007;71:706–710
  22. Eckert DE, Jonitlis AJ, Davidson AJ. The incidence and manifestation of urographic papillary abnormalities in patients with S hemoglobinopathies. Radiology. 1974;113:59–63
  23. Pallone TL, Turner MR, Edwards A, Jamison RL. Countercurrent exchange in the renal medulla. Am J Physiol Regul Integr Comp Physiol. 2003;284:R1153–R1175
  24. McInnes BK. The management of hematuria associated with sickle hemoglobinopathies. J Urol. 1980;124:171–174
  25. Gupta AK, Kirchner KA, Nicholson R, et al. Effects of alpha thalassemia and sickle polymerization tendency on the urine-concentrating defects of individuals with sickle cell trait. J Clin Invest. 1991;88:1963–1968
  26. Diggs LW. The sickle cell trait in relation to the training and assignment of duties in the armed forces: III (Hyposthenuria, hematuria, sudden death, rhabdomyolysis and acute tubular necrosis). Aviat Space Environ Med. 1984;55:358–364
  27. Franlin QJ, Compeggie M. Splenic syndrome in sickle cell trait: four case presentations and a review of the literature. Mil Med. 1999;164:230–233
  28. Cooley JC, Peterson WL, Engel CE, Jernigan JP. Clinical triad of massive splenic infarction, sicklemia trait, and high altitude flying. JAMA. 1954;154:111–113
  29. Sheikha A. Splenic syndrome in patients at high altitude with unrecognized sickle cell trait: splenectomy is often unnecessary. Can J Surg. 2005;48:377–381
  30. Mitchell BL. Sickle cell trait and sudden death-bringing it home. J Natl Med Assoc. 2007;99:300–305
  31. Kark JA, Posey DM, Schumacher HR, Ruehle CJ. Sickle cell trait as a risk factor for sudden death in physical training. N Engl J Med. 1987;317:781–787
  32. Drehner D, Neuhauser KM, Neuhauser TS, Blackwood GV. Death among US Airforce basic trainees, 1956 to 1996. Mil Med. 1999;164:841–847
  33. National Athletic Trainers' Association. Consensus Statement: Sickle cell trait and the athlete. http://www.nata.org/statements/consensus/sicklecell.pdf
  34. Allison AC. Protection afforded by sickle cell trait against subtertian malarial infection. Br Med J. 1954;1:290–294
  35. Williams TN, Mwangi TW, Wambna S, et al. Sickle cell trait and the risk of plasmodium malaria and other childhood diseases. J Infect Dis. 2005;192:178–186
  36. Williams TN, Mwangi TW, Roberts DJ, et al. An immune basis for malaria protection by the sickle cell trait. PLos Med. 2005;2:e128;Epub 2005 May 31
  37. Austin H, Key NS, Benson JM, et al. Sickle cell trait and the risk of venous thromboembolism among blacks. Blood. 2007;110:908–912
  38. Heller P, Best WR, Nelson RB, Becktel J. Clinical implications of sickle cell trait and glucose-6-phosphate dehydrogenase deficiency in hospitalized black male patients. N Engl J Med. 1979;300:1001–1005
  39. Westerman MP, Green D, Gilman-Sachs A, et al. Coagulation changes in individuals with sickle cell trait. Am J Hematol. 2002;69:89–94
  40. Taylor MY, Wyatt Ashmead, Gray J, et al. Pregnancy loss after first trimester viability in women with sickle cell trait: time for a re-appraisal?. Am J Obstet Gynecol. 2006;194:1604–1608
  41. Larabee KD, Monga M. Women with sickle cell trait are at increased risk for pre-eclampsia. Am J Obstet Gynecol. 1997;177:425–428
  42. Hooper CY, Fraser-Bell S, Farinelli A, Grigg JR. Complicated hyphema: think sickle. Clin Experiment Opthalmol. 2006;34:377–378
  43. Fany A, Boni S, Adjorlolo C, et al. Retinopathy as a sickle cell trait: myth or reality?. J Fr Opthalmol. 2004;27:1025–1030
  44. Nia J, Lam WC, Kleinman DM, et al. Retinopathy in sickle cell trait: does it exist?. Can J Opthalmol. 2003;38:46–51
  45. Dourakis SP, Alexopoulou C, Kaloterakis A, Hadziyannis SJ. Acute chest syndrome in sickle-cell trait (Two case reports in persons of Mediterranean origin and review of the literature). Eur J Intern Med. 2004;15:248–250
  46. Whalley PJ, Martin FG, Pritchard JA. Sickle cell trait and urinary tract infection during pregnancy. JAMA. 1964;189:903–906
  47. Thurman RT, Steed LL, Hulsey T, Soper DE. Bacteriuria in pregnant women with sickle cell trait. Am J Obstet Gynecol. 2006;194:1336–1370

 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

Article Tools