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ABSTRACT
Objective. Leg ulcer is the most common skin chronic complication sickle cell related. The aim of this study is to report epidemiological, clinical and biological features of adult sickle cell patients with current leg ulcer. Materials and methods. This was a comparative cross-sectional descriptive study carried out over a period of one year (from January 1st to December 31st 2016) in the clinical hematology outpatient department. We analyzed epidemiological, clinical and biological features of adult sickle cell encounters in stable period with leg ulcer. Results. Thirty five patients (24.65%), in stable period, consulted for leg ulcer. Their mean age at the diagnostic was 26.04. The mean duration of the leg ulcer was 21 days. Leg ulcer occurred spontaneously in 77.14% (n=27). Leg ulcer was single in 91.43% (n=32). The most common site was the internal malleolus: 51.43% (n=18). The mean hemoglobin and LDH rate were respectively 7.53 g/dl and 697.71 UI/L. The multivariate analysis found a statistically significant correlation between the level of the LDH and leg ulcer. Twenty seven patients (77.14%) with leg ulcer had high level of LDH. The LDH level was higher in the sickle cell population with leg ulcer than the one without leg ulcer. The hemoglobin level was lower in the population with leg ulcer (7.53 g/dL). P=0.001. Conclusion. High level of LDH, anemia and hyperhemolysis are factors associated with leg ulcer in sickle cell population.
RÉSUMÉ
Objectif. L’ulcère de jambe est la complication chronique de localisation cutanée la plus fréquente chez le sujet drépanocytaire. L’objectif de cette étude est de rapporter les caractéristiques épidémiologiques, cliniques et biologiques du sujet drépanocytaire homozygote ayant un ulcère de jambe actif. Matériels et méthodes. Il s’agit d’une étude transversale descriptive comparative réalisée sur une période de un an (1er janvier 2016 au 31 décembre 2016) dans le service de consultation externe d’Hématologie Clinique du CHU de Brazzaville au Congo. Nous avons analysé les paramètres épidémiologiques, cliniques et biologiques des sujets drépanocytaires âgés de 18 ans et plus en période intercritique consultant pour un ulcère de jambe. Résultats. Trente cinq patients, en période intercritique, ont consulté pour un ulcère de jambe, soit une fréquence de 24,65%. L’âge moyen au diagnostic était de 26,04 ans. La durée moyenne de l’évolution de la vasculopathie avant la consultation était de 21 jours. Le mode de survenue était spontané chez 77,14% (n=27). L’ulcère de jambe était unique dans 91,43% (n=32), situé en regard de la malléole interne chez 18 patients (51,43%). Le taux moyen d’hémoglobine et de la LDH étaient respectivement 7,53g/dl et de 697,71 UI/L. L’analyse multivariée a montré une corrélation statistique significative entre le taux de LDH et la fréquence de l’UJ. Vingt sept patients (77,14%) ayant un taux de LDH ont en effet développé un UJ. En outre, le taux de LDH était plus élevé par rapport à la population témoin. Le taux d’hémoglobine était significativement plus bas dans le groupe de patients ayant développé un ulcère (7,53g/dl). P=0,001. Conclusion.Ces résultats confirment que l’anémie, l’hyperhémolyse et un taux élevé de LDH sont significativement associés à la survenue de l’ulcère de jambe chez le sujet drépanocytaire.
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References
- World Health Organization. Guidelines for the Control Haemoglobin Disorders. Geneva: World Health: 1-10rganization. Hereditary Diseases Program, 1994: 1-32.
- Pauling L, Itano HA, Singer SJ, Wells JC. Sickle cell anemia, a molecular disease. Science 1949; 110: 543-8.
- Redelsperger MM, Bardakdjian-Michau J, Neonato MG, Girot R. Diagnostic biologique des syndromes drépanocytaires. La drépanocytose. John Libbey Eurotext 2003 : 13-30.
- Akinyanju O, Akinsete I. Leg ulceration in sickle cell in Nigeria. Trop Geogr Med 1979; 31: 87-91.
- Koshy M, Entsuah R, Koranda A, et al. Leg ulcers in patients with sickle cell. Blood 1989; 74: 1403-8.
- Ndiaye M, Niang S.O, Diop A, et al. Ulcères de jambe au cours de la drépanocytose : étude rétrospective de 40 cas. Ann de Dermatologie et Vénérologie 2016; 143 : 103-7.
- Serjeant GR. Leg ulceration in homozygous sickle cell disease in Jamaica. International aspects of sickle cell disease. Proceedings of the international conference on sickle cell disease. A world health problem. Washington DC, 1973; 103.
- Mack AK, Kato GJ. Sickle cell disease and nitric oxide: A paradigm shit? In J Biochem and Cell Biol 2006; 38(8): 1237-43.
- Mohan JS, Marshall JM, Reid HL, et al. Postural vasoconstriction and leg ulceration in homozygous sickle cell disease. Clin Sci (Lord) 1997; 92: 153-8.
- Knox-Macaulay HH. Sickle cell in Sierra Leone. A clinical and haematological analysis in older children and adults. Ann Trop Med Parasitol 1983; 77: 411-9.
- Alavi A, Kirsner R. Hemoglobinopathies and leg ulcers. Int L Low Extrem Wounds 2015; 14(3): 213-6.
- Delaney KMH, Axelrod KC, Buscetta A, et al .Leg ulcers in sickle cell disease: current patterns and practice. Hemoglobin 2013; 37: 325-32.
- Nolan VG, Adewoye A, Baldwin C, et al. Sickle cell ulcers: associations with haemolysis and SNPs in Klotho, TEK genes of the TGF-β/BMP pathway. BJH 2006; 133(5): 570-78.
- Minniti CP, Eckman J, Sebastiani P, et al Leg ulcers in sickle cell disease AM J of Hematology 2010; 85(10):831-3.
- Kato JK, Gladwin MT, Steinberg MH et, al. Deconstructiong sickle cell: reappraisal of the role of the hemolysis in the development of clinical subphenotypes. Blood reviews 2007; 21(1):37-47.
- Kato JK, McGowan V, Machado RF,et al. Lactate dehydrogenase as biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension and death in patients with Sickle Cell Disease. Blood 2006; 107: 2279-85.
- Ballas SK. Lactate dehydrogenase and hemolysis in sickle cell disease. Blood 2013; 121: 243-44.
- Dubert M, Menet A, Tolo A, et al. Association entre hyperhémolyse chronique et les complications vasculaire de la drépanocytose en Afrique Subsaharienne. Le Revue de Médecine Interne ; 36, A95.
References
World Health Organization. Guidelines for the Control Haemoglobin Disorders. Geneva: World Health: 1-10rganization. Hereditary Diseases Program, 1994: 1-32.
Pauling L, Itano HA, Singer SJ, Wells JC. Sickle cell anemia, a molecular disease. Science 1949; 110: 543-8.
Redelsperger MM, Bardakdjian-Michau J, Neonato MG, Girot R. Diagnostic biologique des syndromes drépanocytaires. La drépanocytose. John Libbey Eurotext 2003 : 13-30.
Akinyanju O, Akinsete I. Leg ulceration in sickle cell in Nigeria. Trop Geogr Med 1979; 31: 87-91.
Koshy M, Entsuah R, Koranda A, et al. Leg ulcers in patients with sickle cell. Blood 1989; 74: 1403-8.
Ndiaye M, Niang S.O, Diop A, et al. Ulcères de jambe au cours de la drépanocytose : étude rétrospective de 40 cas. Ann de Dermatologie et Vénérologie 2016; 143 : 103-7.
Serjeant GR. Leg ulceration in homozygous sickle cell disease in Jamaica. International aspects of sickle cell disease. Proceedings of the international conference on sickle cell disease. A world health problem. Washington DC, 1973; 103.
Mack AK, Kato GJ. Sickle cell disease and nitric oxide: A paradigm shit? In J Biochem and Cell Biol 2006; 38(8): 1237-43.
Mohan JS, Marshall JM, Reid HL, et al. Postural vasoconstriction and leg ulceration in homozygous sickle cell disease. Clin Sci (Lord) 1997; 92: 153-8.
Knox-Macaulay HH. Sickle cell in Sierra Leone. A clinical and haematological analysis in older children and adults. Ann Trop Med Parasitol 1983; 77: 411-9.
Alavi A, Kirsner R. Hemoglobinopathies and leg ulcers. Int L Low Extrem Wounds 2015; 14(3): 213-6.
Delaney KMH, Axelrod KC, Buscetta A, et al .Leg ulcers in sickle cell disease: current patterns and practice. Hemoglobin 2013; 37: 325-32.
Nolan VG, Adewoye A, Baldwin C, et al. Sickle cell ulcers: associations with haemolysis and SNPs in Klotho, TEK genes of the TGF-β/BMP pathway. BJH 2006; 133(5): 570-78.
Minniti CP, Eckman J, Sebastiani P, et al Leg ulcers in sickle cell disease AM J of Hematology 2010; 85(10):831-3.
Kato JK, Gladwin MT, Steinberg MH et, al. Deconstructiong sickle cell: reappraisal of the role of the hemolysis in the development of clinical subphenotypes. Blood reviews 2007; 21(1):37-47.
Kato JK, McGowan V, Machado RF,et al. Lactate dehydrogenase as biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension and death in patients with Sickle Cell Disease. Blood 2006; 107: 2279-85.
Ballas SK. Lactate dehydrogenase and hemolysis in sickle cell disease. Blood 2013; 121: 243-44.
Dubert M, Menet A, Tolo A, et al. Association entre hyperhémolyse chronique et les complications vasculaire de la drépanocytose en Afrique Subsaharienne. Le Revue de Médecine Interne ; 36, A95.