Úlceras de perna em pacientes com anemia falciforme
Leg ulcers in sickle cell disease patients
Paula Dadalti Granja; Samuel Braulio Magalhães Quintão; Franciele Perondi; Rosemary Bacellar Ferreira de Lima; Cláudia Labriola de Medeiros Martins; Marcos Arêas Marques; Julio Cesar Peclat de Oliveira
Leg ulcers are the most common cutaneous complication of sickle cell disease. These lesions occur mainly in homozygous forms, are slow to heal and often relapse, causing negative physical, emotional, and economic impacts. In this paper, we discuss the clinical presentation, diagnosis, and pathophysiology of sickle cell leg ulcers and their implications for treatment.
1 Minniti CP, Eckman J, Sebastiani P, Steinberg MH, Ballas SK. Leg Ulcers in Sickle Cell Disease. Am J Hematol. 2010;85(10):831-3.
2 Aldallal SM. Mini review: leg ulcers - a secondary complication of sickle cell disease. Int J Gen Med. 2019;12:279-82.
3 Meneses JVL, Ribeiro IIF, Guedes A, et al. Úlceras maleolares em portadores de anemia falciforme: manejo clínico e operatório. Gaz. Med. 2010;80(3):89-94.
4 Minniti CP, Kato GJ. How we treat sickle cell patients with leg ulcers. Am J Hematol. 2016;91(1):22-30.
5 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(6):1315-24.
6 Umeh NI, Ajegba B, Buscetta AJ, Abdallah KE, Minniti CP, Bonham VL. The psychosocial impact of leg ulcers in patients with sickle cell disease: I don’t want them to know my little secret. PLoS One. 2017;12(10):e0186270.
7 Brasil. Secretaria de Atenção à Saúde. Departamento de Atenção Especializada. Doença Falciforme - úlceras prevenção e tratamento. Série B: Textos Básicos de Saúde. Brasília: Ministério da Saúde, 2012.
8 Serjeant GR, Serjeant BE, Mohan JS, Clare A. Leg ulceration in sickle cell disease: medieval medicine in a modern world. Hematol Oncol Clin North Am. 2005;19(5):943-56.
9 Antwi-Boasiako C, Campbell AD. Low Nitric oxide level is implicated in sickle cell disease and its complications in Ghana. Vasc Health Risk Manag. 2018;14:199-204.
10 Minniti CP, Delaney KM, Gorbach AM, et al. Vasculopathy, inflammation and blood flow in leg ulcers of patients with sickle cell anemia. Am J Hematol. 2014;89(1):1-6.
11 Domingos IF, Pereira-Martins DA, Sobreira MJVC, et al. High Levels of Proinflammatory Cytokines IL-6 and IL-8 are associated with a poor clinical outcome in sickle cell anemia. Ann Hematol. 2020;99(5):947-53.
12 Morton LM, Phillips TJ. Wound healing and treating wounds. Differential diagnosis and evaluation of chronic wounds. J Am Acad Dermatol. 2016;74(4):589-605.
13 Kendall C. Sickle cell leg ulcer: a case study. Plast Surg Nurs. 2018;38(3):99-100.
14 Morgante A, Li Destri A. Skin ulcers complicating sickle cell disease: an interlinked reparative model. G Chir. 2019;40(5):441-4. PMid:32003727.
15 Ladizinski B, Bazakas A, Mistry N, Alavi A, Sibbald RG, Salcido R. Sickle cell disease and leg ulcers. Adv Skin Wound Care. 2012;25(9):420-8.
16 Wethers DL, Ramirez GM, Koshy M, et al. Accelerated healing of chronic sickle-cell leg ulcers treated with RGD peptide matrix. RGD Study Group. Blood. 1994;84(6):1775-9.
17 Martí-Carvajal AJ, Knight-Madden JM, Martinez-Zapata MJ. Interventions for treating leg ulcers in people with sickle cell disease. Cochrane Database Syst Rev. 2014;2014(12):CD008394. PMid:25485858.
18 Bonini-Domingos CR, Valente FM. Low level laser therapy of leg ulcer in sickle cell anemia. Rev Bras Hematol Hemoter. 2012;34(1):65-6.
19 Nicolaides AN. The most severe stage of chronic venous disease: An update of management of patients with venous leg ulcers. Adv Ther. 2020;37(S1, Suppl 1):S19-24.
20 Frost ML, Treadwell P. Treatment of sickle cell leg ulcers with pentoxifillyne. Int J Dermatol. 1990;29(5):375-6.
21 Antonioli E, Guglielmelli P, Pieri L, et al. Hydroxyurea-related toxicity in 3,411 patients with Ph negative MPN. Am J Hematol. 2012;87(5):552-4.
22 Minniti CP, Gorbach AM, Xu D, et al. Topical sodium nitrite for chronic leg ulcers in patients with sickle cell anemia. A phase 1 dose-finding safety and tolerability trial. Lancet Haematol. 2014;1(3):x95-e103.
23 Connor JL Jr, Sclafani JA, Kato GJ, Hsieh MM, Minniti CP. Brief topical sodium nitrite and its impact on the quality of life in patients with sickle cell ulcers. Medicine (Baltimore). 2018;97(46):1-5.
24 Eckman JR. Leg ulcers in sickle cell disease. Hematol Oncol Clin North Am. 1996;10(6):1333-44.
25 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(5):1154-61.
26 Alden PB, Lips EM, Zimmerman KP, et al. Chronic venous ulcer: minimally invasive treatment of superficial axial and perforator vein reflux speeds healing and reduces recurrence (comparative study). Ann Vasc Surg. 2013;27(1):75-83.
27 Ayoola OO, Bolarinwa RA, Onakpoya UU, Adedeji TA, Onwuka CC, Idowu BM. Intima-media thickness os the common femoral artery as a marker of leg ulceration in sickle cell disease patients. Blood Adv. 2018;5(23):3112-7.
28 Singh AP, Minniti CP. Leg ulceration in sickle cell disease: an early and visible sign of end-organ disease. In: Inusa BPD, editor. Sickle Cell Disease: Pain and common chronic complications. London, UK: InTech; 2016. p. 171-202.