Sickle cell disease induces resistance to cutaneous carcinogenesis

• Published in: Orphanet journal of rare diseases Vol. 15; no. 1; pp. 66 - 5
• Main Authors: Soutou, Boutros, Senet, Patricia, Lionnet, François, Habibi, Anoosha, Aractingi, Sélim
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 06.03.2020; BioMed Central; BMC
• Subjects: Actinic keratosis; Age; Cancer; Carcinogenesis; Carcinoma; Care and treatment; Dermatology; Disease resistance; Diseases; Drug dosages; Hematology; Hemoglobin; Human health and pathology; Hydroxyurea; Keratosis; Leg ulcers; Life Sciences; Medical prognosis; Medical research; Melanoma; Mutation; Rare diseases; Santé publique et épidémiologie; Sickle cell anemia; Sickle cell disease; Skin; Skin cancer; Skin diseases; Skin ulcer; Tumors; Ulcers; France; Sub-Saharan Africa; Africa; West Indies; Hydroxyurea; Sickle cell disease; Skin ulcer; Skin cancer
• ISSN: 1750-1172; 1750-1172
• DOI: 10.1186/s13023-020-1341-9

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. In 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. This 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.