Skip to main content
SpringerLink
Log in

Sickle Cell Anemia and Ulcer

  • Chapter
  • First Online:
Uncommon Ulcers of the Extremities

  • 135 Accesses

Abstract

Sickle cell hemoglobin (HbS) is a variant of adult hemoglobin due to mutation of beta chain of globin, which leads to substitution of valine amino acid for glutamic acid at sixth position of beta globin (base pair change: thymine for adenine at sixth codon of β-globin gene). Sickle cell disease refers to any condition in which the production of HbS causes pathophysiological consequences as a result of homozygous inheritance of βS-mutation and referred as either SCD SS or SCA. It includes Sickle cell anemia (SCA), which is the most severe form of the disease but also the compound heterozygotes, where one allele includes sickle cell mutation and second allele includes gene mutation other than the sickle cell mutation, such as HbC, beta thalassemia, HbD, and HbO [1, 2]. Homozygous SS patients are the ones most likely associated with ulcerations.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 119.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 159.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Rees DC, Williams TN, Gladwin MT. Sickle cell disease. Lancet. 2010;376:2018–31.

    Article  CAS  PubMed  Google Scholar 

  2. Powars DR, Chan LS, Hiti A, Ramicone E, Johnson C. Outcome of sickle cell anemia: a 4-decade observational study of 1056 patients. Medicine (Baltimore). 2005;84:363–76.

    Article  PubMed  Google Scholar 

  3. Parent F, Bachir D, Inamo J, et al. A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med. 2011;365:44–53.

    Article  CAS  PubMed  Google Scholar 

  4. Minniti CP, Eckman J, Sebastiani P, Steinberg MH, Ballas SK. Leg ulcers in sickle cell disease. Am J Hematol. 2010;85:831–3.

    Article  PubMed  PubMed Central  Google Scholar 

  5. Nouraie M, Lee JS, Yingze ZY. The relationship between the severity of hemolysis, clinical manifestations and risk of death in 415 patients with sickle cell anemia in the US and Europe. Haematologica. 2013;98:464–72.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Bunn HF, Nathan DG, Dover GJ, et al. Pulmonary hypertension and nitric oxide depletion in sickle cell disease. Blood. 2010;116:687e92.

    Article  Google Scholar 

  7. Nguyen VT, Nassar D, Batteux F, et al. Delayed healing of sickle cell ulcers is due to impaired angiogenesis and CXCL12 secretion in skin wounds. J Invest Dermatol. 2016;136:497–506.

    Article  CAS  PubMed  Google Scholar 

  8. Monfort JB, Senet P. Leg ulcers in sickle-cell disease: treatment update. Adv Wound Care. 2020;9(6):348–56.

    Article  Google Scholar 

  9. Serjeant GR. Leg ulceration in sickle cell anemia. Arch Int Med. 1974;133:690–4.

    Article  CAS  Google Scholar 

  10. Altman IA, Kleinfelder RE, Quigley JG, Ennis WJ, Minniti CP. A treatment algorithm to identify therapeutic approaches for leg ulcers in patients with sickle cell disease. Int Wound J. 2016;13:1315–24.

    Article  PubMed  Google Scholar 

  11. Stevens DM, Shupack JL, Javid J, Silber R. Ulcers of the leg in thalassemia. Arch Dermatol. 1977;113:1558–60.

    Article  CAS  PubMed  Google Scholar 

  12. Wethers DL, Ramirez GM, Koshy M, Steinberg MH, Phillips G, Siegel RS, et al. Accelerated healing of chronic sickle-cell leg ulcers treated with RGD peptide matrix. RGD Study Group. Blood. 1994;84:1775–9.

    Article  CAS  PubMed  Google Scholar 

  13. Minniti CP, Gorbach AM, Xu D, Hon YY, Delaney KM, Seidel M, et al. Topical sodium nitrite for chronic leg ulcers in patients with sickle cell anaemia: a phase 1 dose-finding safety and tolerability trial. Lancet Haematol. 2014;1:e95–103.

    Article  PubMed  PubMed Central  Google Scholar 

  14. La Grenade L, Thomas PW, Serjeant GR. A randomized controlled trial of solcoseryl and duoderm in chronic sickle-cell ulcers. West Indian Med J. 1993;42:121–3.

    PubMed  Google Scholar 

  15. Garwood CS, Kim PJ, Matai V, et al. The use of bovine collagen-glycosaminoglycan matrix for atypical lower extremity ulcers. Wounds. 2016;28:298–305.

    PubMed  Google Scholar 

  16. Reindorf CA, Walker-Jones D, Adekile AD, Lawal O, Oluwole SF. Rapid healing of sickle cell leg ulcers treated with collagen dressing. J Natl Med Assoc. 1989;81:866–8.

    CAS  PubMed  PubMed Central  Google Scholar 

  17. Alikhan MA, Carter G, Mehta P. Topical GM-CSF hastens healing of leg ulcers in sickle cell disease. Am J Hematol. 2004;76:192.

    Article  PubMed  Google Scholar 

  18. Serjeant GR, Galloway RE, Gueri MC. Oral zinc sulphate in sickle-cell ulcers. Lancet. 1970;2:891–2.

    Article  CAS  PubMed  Google Scholar 

  19. Frost ML, Treadwell P. Treatment of sickle cell leg ulcers with pentoxifylline. Int J Dermatol. 1990;29:375–6.

    Article  CAS  PubMed  Google Scholar 

  20. Eckman JR. Leg ulcers in sickle cell disease. Hematol Oncol Clin North Am. 1996;10:1333–44.

    Article  CAS  PubMed  Google Scholar 

  21. Serjeant GR, Howard C. Isoxsuprine hydrochloride in the therapy of sickle cell leg ulceration. West Indian Med J. 1977;26:164–6.

    CAS  PubMed  Google Scholar 

  22. Voskaridou E, Christoulas D, Bilalis A, et al. The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS). Blood. 2010;115:2354–63.

    Article  CAS  PubMed  Google Scholar 

  23. Lanzkron S, Strouse JJ, Wilson R, et al. Systematic review: hydroxyurea for the treatment of adults with sickle cell disease. Ann Intern Med. 2008;148:939–55.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Ashby RL, Gabe R, Ali S, et al. Clinical and cost-effectiveness of compression hosiery versus compression bandages in treatment of venous leg ulcers (venous leg ulcer study IV, VenUS IV): a randomized controlled trial. Lancet. 2014;383:871–9.

    Article  PubMed  Google Scholar 

  25. Barwell JR, Davies CE, Deacon J, et al. Comparison of surgery and compression with compression alone in chronic venous ulceration (ESCHAR study): randomised controlled trial. Lancet. 2004;363:1854–9.

    Article  PubMed  Google Scholar 

  26. Weinzweig N, Schuler J, Marschall M, Koshy M. Lower limb salvage by microvascular free-tissue transfer in patients with homozygous sickle cell disease. Plast Reconstr Surg. 1995;96:1154–61.

    Article  CAS  PubMed  Google Scholar 

  27. Mauck KF, Asi N, Undavalli C, et al. Systematic review and meta-analysis of surgical interventions versus conservative therapy for venous ulcers. J Vasc Surg. 2014;60:60S–70S.

    Article  PubMed  Google Scholar 

  28. Gohel MS, Heatley F, Liu X, et al. A randomized trial of early endovenous ablation in venous ulceration. N Engl J Med. 2018;378:2105–14.

    Article  PubMed  Google Scholar 

  29. Holm JS, Toyserkani NM, Sorensen JA. Adipose-derived stem cells for treatment of chronic ulcers: current status. Stem Cell Res Ther. 2018;9:142.

    Article  PubMed  PubMed Central  Google Scholar 

  30. Burgos-Alonso N, Lobato I, Hernández I, et al. Adjuvant biological therapies in chronic leg ulcers. Int J Mol Sci. 2017;18:E2561.

    Article  Google Scholar 

  31. Meneses JV, Fortuna V, de Souza ES, et al. Autologous stem cell-based therapy for sickle cell leg ulcer: a pilot study. Br J Haematol. 2016;175:949–55.

    Article  PubMed  Google Scholar 

  32. Connor JL Jr, Minniti CP, Tisdale JF, Hsieh MM. Sickle cell anemia and comorbid leg ulcer treated with curative peripheral blood stem cell transplantation. Int J Low Extrem Wounds. 2017;16:56–9.

    Article  PubMed  PubMed Central  Google Scholar 

  33. White J, Ivins N, Wilkes A, Carolan-Rees G, Harding KG. Non-contact low-frequency ultrasound therapy compared with UK standard of care for venous leg ulcers: a single-centre, assessor-blinded, randomised controlled trial. Int Wound J. 2016;13:833–42.

    Article  PubMed  Google Scholar 

  34. Dumville JC, Land L, Evans D, Peinemann F. Negative pressure wound therapy for treating leg ulcers. Cochrane Database Syst Rev. 2015;7:CD011354.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Paediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India

    Rajniti Prasad & Meenakshi Pandey

Authors
  1. Rajniti Prasad
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Meenakshi Pandey
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Institute of Medical Sciences, Department of Surgery, Banaras Hindu University, Varanasi, India

    Ajay K. Khanna

  2. Institute of Medical Sciences, Department of General Surgery, Banaras Hindu University, Varanasi, India

    Satyendra Kumar Tiwary

Rights and permissions

Reprints and permissions

Copyright information

© 2023 The Author(s), under exclusive license to Springer Nature Singapore Pte Ltd.

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Prasad, R., Pandey, M. (2023). Sickle Cell Anemia and Ulcer. In: Khanna, A.K., Tiwary, S.K. (eds) Uncommon Ulcers of the Extremities. Springer, Singapore. https://doi.org/10.1007/978-981-99-1782-2_18

Download citation

  • DOI: https://doi.org/10.1007/978-981-99-1782-2_18

  • Published:

  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-99-1781-5

  • Online ISBN: 978-981-99-1782-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation