Learning By images

einstein. 2009;7(2 Pt 1):243-4

Ulcers related to sickle cell anemiaLesão ulcerativa relacionada à anemia falciforme

Rodrigo Damião Maia Graciano*

Sickle cell anemia is one of the most common genetic diseases and was first described by Herrick(1), in 1910. It is frequent but not exclusive in black individuals, and originates from the replacement of the amino acid valine (Val) by glutamine (Glu) at position 6 in the beta globin chain, resulting in hemoglobin S. In hypoxic conditions, the erythrocytes with predominantly hemoglobin S (HbS) take the shape of a sickle – hence the name of the disease – due to polymerization of hemoglobin S(2-3).

The hemoglobin S gene is highly frequent in tropical Africa and among black people from countries involved in slavery traffic(4). In Brazil, approximately 0.1 to 0.3% of the black population is affected by the disease(5-6) and it is estimated that at least two million individuals are HbS carriers (heterozygotes). The clinical manifestations of the disease are present as from the first year throughout life and vary much(7).

One of the complications of sickle cell anemia is leg ulcers, which usually initiate as lesions that are small

or related to external factors. The ulcers are difficult to treat and heal, and require a perfect and coordinated cascade of cell and molecular events interacting to result in reconstruction of tissues(8). Such events comprise a dynamic process, involving biochemical and physiologic phenomena that act in a harmonious manner to ensure tissue restoration (Figure 1A and B).

The ulcers have a significant social and economic impact due to their recurrent nature and to the prolonged period between onset and healing. Hence, due to the need of prolonged therapies, ulcer patients often require care delivered by diverse healthcare professionals, they are frequently on sick leave and usually retire earlier(9). All these factors result in a marked burden to health and welfare systems due to high costs of treatment, absenteeism and unemployment, in addition to interfering in the quality of life of patients from having less pleasant daily activities.

Study carried out at Hospital Israelita Albert Einstein – HIAE, São Paulo (SP), Brazil.

* Nurse specialist in Intense Care Nursing; Postgraduate student in Neurology at Faculdade de Enfermagem of Hospital Israelita Albert Einstein – HIAE, São Paulo (SP), Brazil.

Corresponding author: Rodrigo Damião Maia Graciano – Rua Araucária, 23 – Jardim França – CEP 02338-010 – São Paulo (SP), Brasil – Tel.: 11 7160-5618 – e-mail: rodrigo_graciano@hotmail.com

Received on: Nov 12, 2008 – Accepted on: Feb 16, 2009

Figure 1. (A) A 21-year old black patient with an ulcer in the left lower limb: flat, irregular edges, significant amount of fibrin and areas of granulation tissue; (B) increase of the granulation tissue

a B

einstein. 2009;7(2 Pt 1):243-4

244 Graciano RDM

reFerenCes 1. Gòmez-Chiari M, Puigbert JT, Aramburu JO. Drepanocitosis: experiência de

um centro. An Pediatr. 2003;58:95-9.

2. Serjeant GR. A doença da célula falciforme. Anais Nestlè. 1999;58:11-22.

3. Costa FF. Anemia falciforme. In: Zago MA, Falcão RP, Pasquini R. Hematologia – fundamentos e prática. Rio de Janeiro: Atheneu; 2001. p 289-307.

4. Wang WC, Lukens JN. Sickle cell anemia and other sickling syndromes. In: Lee GR, Foerster J, Lukens J, Paraskevas F, Greer JP, Rodgers GM, editors. Wintrobes clinical hematology. Baltimore (MD): Williams & Wilkins; 1999. p. 1346-97.

5. Naoum PC, Alvarez FF, Domingos CRB, Ferrari F, Moreira HW, Sampaio Z, et al. Hemoglobinas anormais no Brasil: prevalência e distribuição geográfica. Rev Bras Patol Clin. 1987;23(3):68-79.

6. Ramalho AS, Magna LA, Paiva e Silva RB. A portaria nº 822/01 do Ministério da Saúde e as peculiaridades das hemoglobinopatias em saúde pública no Brasil. Cad Saúde Pública. 2003;19(4):1195-9.

7. Ministério da Saúde. Agência Nacional de Vigilância Sanitária. Manual de diagnóstico e tratamento de doenças falciformes. Brasília (DF): Anvisa; 2002. p. 9-11.

8. Ortonne JP, Clév JP. Physiologie de La cicatrisation cutanée. Rev Prat. 1994;44(13):1733-4.

9. Abbade LP, Lastória S, de Almeida Rollo H, Stolf HO. A sociodemographic, clinical study of patients with venous ulcer. Int J Dermatol. 2005;44(12): 989-92.