Abstract
BackgroundWhile skin carcinomas are reported in chronic ulcers and in patients treated with hydroxyurea (HU) for myeloproliferative neoplasms, no skin carcinoma has been reported in patients with sickle cell disease (SCD), presenting chronic skin ulcers or treated with HU. The objective was to estimate the risk of cutaneous malignant transformation in SCD patients with prolonged leg ulcers or under HU therapy.ResultsIn this cross-sectional study, the cohort consisted of 1543 patients. In the first series, 29 patients presented a total of 53 ulcers lasting more than two years. The median age was 35 ± 8.4 years old. The median duration for a single ulcer was 9.2 ± 7 years. None of the examined ulcers showed any suspicious area of malignant transformation. In the second series, 187 patients treated with HU for more than two years were identified. The median age was 31.3 ± 9.9 years old. The median duration of treatment with HU was 6 ± 3.2 years. No skin carcinoma or actinic keratosis was recorded.ConclusionsThis study showed that skin carcinogenesis did not occur in our series of SCD patients exposed to transforming events such as long term HU treatment or prolonged leg ulcers.
Highlights
While skin carcinomas are reported in chronic ulcers and in patients treated with hydroxyurea (HU) for myeloproliferative neoplasms, no skin carcinoma has been reported in patients with sickle cell disease (SCD), presenting chronic skin ulcers or treated with HU
The aim of this study was to estimate the risk of cutaneous malignant transformation in patients with SCD presenting any of the classical carcinogenic events, namely Chronic leg ulcers (CLU) or HU treatment
The cohort of SCD patients followed in the two referral centers at the time of the study consisted of 1543 patients, 1078 displaying the SS genotype from which we selected the SCD-prolonged ulcer group that corresponded to 29 patients presenting a total of 53 ulcers non healing ulcers for at least 2 years
Summary
While skin carcinomas are reported in chronic ulcers and in patients treated with hydroxyurea (HU) for myeloproliferative neoplasms, no skin carcinoma has been reported in patients with sickle cell disease (SCD), presenting chronic skin ulcers or treated with HU. The objective was to estimate the risk of cutaneous malignant transformation in SCD patients with prolonged leg ulcers or under HU therapy. Sickle cell disease (SCD) is a genetic disorder of hemoglobin secondary to a mutation in the β-globin gene. It affects patients of African heritage classically living in Africa, Caribbean Islands or the Middle East. SCD became the most frequent genetic disease in France and UK [1, 2]. In the U.S.A., the number of individuals with SCD may approach 100,000 [3]
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