Leg ulcer induced by hydroxycarbamide in sickle cell disease: What is the therapeutic impact?

Soya, E.; Makowski, Caroline; Blaise, S.

doi:10.1111/iwj.13115

Cited by 6 publications

(7 citation statements)

References 30 publications

(53 reference statements)

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“…7 This may also imply that therapeutic interventions targeted against leucocytes (including expression of their adhesion molecules) may not successfully reduce the prevalence of this complication, as has been reported with hydroxycarbamide. 24 However, variations in absolute numbers may not represent consequent variations in function or degree of expression of surface molecules as had been noted in SCA.…”

Section: Discussionmentioning

confidence: 99%

Phenotypic characterisation and associations of leg ulcers in adult sickle cell patients

Madu

Madu²,

Anigbogu

et al. 2021

Wound Repair Regeneration

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Sickle leg ulcer (SLU) occurs as a result chronic occlusion of the vasculature with consequent necrosis of the skin and subcutaneous tissue usually in proximity of the malleoli. The description of clinical associations and the simultaneous occurrence of SLU and other complications of SCD compared to the non‐SLU patients was the aim of this work. A total of 272 (60.8% males and 39.2% females) patients were captured during this time period out of which 68 (51 males and 17 females) had SLU out of whom 20 patients had bilateral leg ulcers. Prevalence of SLU was 25% and the median age of patients was 25 years, frequency of crisis 2 per annum and 44 (74.6%) had been transfused in the past. Median Hb of the group was 7.6 g/dl and 25% had values lower than 6.5. The occurrence of other complications in SLU patients was as follows; 10 had AVN, 9 priapism, 8 had osteomyelitis, 6 nephropathy, stroke 2, osteoarthritis 4 and cholelithiasis 4. There was a significant relationship between the occurrence of SLU and gender of the patient being more in 67males—Likelihood ratio 4.610 (p = 0.032) and the occurrence of pulmonary hypertension—Likelihood ratio 4.762 (p = 0.029). There was no significant association between the occurrence of SLU and other complications of SCD. Leg ulcer patients have a median age of 25 years with a prevalence of 25% but do not necessarily show other features of severe disease phenotypes. SLU patients were more likely to develop pulmonary hypertension. Further studies on impact of environmental factors on the occurrence of SLU are needed to further evaluate its aetiology.

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Section: Discussionmentioning

confidence: 99%

Phenotypic characterisation and associations of leg ulcers in adult sickle cell patients

Madu

Madu²,

Anigbogu

et al. 2021

Wound Repair Regeneration

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“…[60][61][62][63] Soya et al reported a case of an SCD patient who developed a leg ulcer 18 days after the introduction of HU and healed after HU was stopped. 64 The authors proposed that the accumulation of HU under the skin could have caused cutaneous atrophy and impaired microcirculation. 64 An alternate hypothesis could be that the level of HbF reached during HU treatment was not enough to provide clinical benefits.…”

Section: Haemolysismentioning

confidence: 99%

Controversies in the pathophysiology of leg ulcers in sickle cell disease

Catella,

Guillot,

Nader

et al. 2024

Br J Haematol

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SummaryPatients with sickle cell disease (SCD) often experience painful vaso‐occlusive crises and chronic haemolytic anaemia, as well as various acute and chronic complications, such as leg ulcers. Leg ulcers are characterized by their unpredictability, debilitating pain and prolonged healing process. The pathophysiology of SCD leg ulcers is not well defined. Known risk factors include male gender, poor social conditions, malnutrition and a lack of compression therapy when oedema occurs. Leg ulcers typically start with spontaneous pain, followed by induration, hyperpigmentation, blister formation and destruction of the epidermis. SCD is characterized by chronic haemolysis, increased oxidative stress and decreased nitric oxide bioavailability, which promote ischaemia and inflammation and consequently impair vascular function in the skin. This cutaneous vasculopathy, coupled with venostasis around the ankle, creates an ideal environment for local vaso‐occlusive crises, which can result in the development of leg ulcers that resemble arterial ulcers. Following the development of the ulcer, healing is hindered as a result of factors commonly observed in venous ulceration, including venous insufficiency, oedema and impaired angiogenesis. All of these factors are modulated by genetic factors. However, our current understanding of these genetic factors remains limited and does not yet enable us to accurately predict ulceration susceptibility.

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“…31 The lesions associated with HC are most often malleolar and have a fibrotic appearance, with inflammatory margins but without necrosis. 32…”

Section: Pathophysiologymentioning

confidence: 99%

“…Hydroxycarbamide can alter microcirculation and cause anoxia of the tissue, which explains the incidence of skin ulcers following minimal trauma and their sometimes painful nature 31 . The lesions associated with HC are most often malleolar and have a fibrotic appearance, with inflammatory margins but without necrosis 32 …”

Section: Pathophysiologymentioning

confidence: 99%

Common, But Neglected: A Comprehensive Review of Leg Ulcers in Sickle Cell Disease

Sahu

Verma

Ganguly

et al. 2021

Adv Skin Wound Care

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OBJECTIVE To compile available evidence to better understand the management of leg ulcers in sickle cell disease (SCD), as well as describe potential therapeutic steps that may be required to improve the quality of life of patients with SCD leg ulcers. DATA SOURCES MEDLINE, PubMed, EMBASE, and Web of Science databases STUDY SELECTION A comprehensive search was conducted to retrieve relevant studies using the keywords “sickle cell disease and leg ulcer,” “ulcer treatments, diagnosis and sickle cell,” and “wound sickle cell.” Studies published through July 2020 were included. DATA EXTRACTION Two independent authors selected all studies that assessed the relationship between leg ulcer and SCD identified from online databases. DATA SYNTHESIS The authors have summarized updated information on pathophysiology (vasculopathy linked to chronic hemolysis and endothelial dysfunction), diagnosis, and available treatment options to unravel the dermohematologic connection between leg ulcers and SCD. CONCLUSIONS It is the authors’ hope that this detailed discussion of the information available on leg ulcers and SCD will lead to a better appreciation of this clinical problem by the clinicians and researchers and in turn have a long-term positive effect on the quality of life of patients with SCD. Researchers should design new trials considering these insights and potential therapeutic approaches based on current knowledge.

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Leg ulcer induced by hydroxycarbamide in sickle cell disease: What is the therapeutic impact?

Cited by 6 publications

References 30 publications

Phenotypic characterisation and associations of leg ulcers in adult sickle cell patients

Phenotypic characterisation and associations of leg ulcers in adult sickle cell patients

Controversies in the pathophysiology of leg ulcers in sickle cell disease

Common, But Neglected: A Comprehensive Review of Leg Ulcers in Sickle Cell Disease

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