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 4 publications

(4 citation statements)

References 30 publications

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“…This further supports the fact that SLU is not peculiar to patients with severe disease or worse phenotypes 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: Discussionsupporting

confidence: 71%

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: Discussionsupporting

confidence: 71%

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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“…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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“…Nevertheless, there are anecdotes of leg ulcers occurring after therapy with HU and of healed old ulcers reactivated after HU therapy. 83 de Montalembert et al followed a cohort of 101 children with SCD treated with HU for a median of 22 months; among these only one 18 year-old patient had leg ulcers 23 months after treatment. 84…”

Section: Bmentioning

confidence: 99%

The Evolving Pharmacotherapeutic Landscape for the Treatment of Sickle Cell Disease

Ballas¹

2020

Mediterr J Hematol Infect Dis

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Sickle cell disease (SCD) is an extremely heterogeneous disease that has been associated with global morbidity and early mortality. More effective and inexpensive therapiesare needed. During the last five years the landscape of the pharmacotherapy of SCD has changed dramatically. Currently, there are at least 50 drugs that have been used or under consideration to use for the treatment of SCD. These fall into 3 categories: the first category includes the three drugs (Hydroxyurea, L-Glutamine and Crizanlizumab tmca) that have been approved by the United States Food and Drug Administration (FDA) based on successful clinical trials. The second category includes 22 drugs that failed, discontinued or terminated for now and the third category includes 25 drugs that are actively being considered for the treatment of SCD. New therapies targeting multiple pathways in its complex pathophysiology have been achieved or are under continued investigation. The emerging trend seems to be the use of multimodal drugs (i.e. drugs that have different mechanisms of action) to treat SCD similar to the use of multiple chemotherapeutic agents to treat cancer.

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

Cited by 4 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

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

The Evolving Pharmacotherapeutic Landscape for the Treatment of Sickle Cell Disease

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