Details

Autor(en) / Beteiligte

• Santos, Edvan do Carmo
• Santana, Paulo Vinícius Bispo
• Jesus, Laíne Lopes Silva de
• Melo, Gabriela Imbassahy Valentim
• Yahouédéhou, Sètondji Cocou Modeste Alexandre
• Guarda, Caroline Conceição da
• Santiago, Rayra Pereira
• Fiuza, Luciana Magalhães
• Carvalho, Suéllen Pinheiro
• Santos, Liz Oliveira Dos
• Adorno, Elisângela Vitória
• Aleluia, Augusto Cezar Magalhães
• Luiz, Luciene Cristina Gastalho Campos
• Fonseca, Teresa Cristina Cardoso
• Gonçalves, Marilda de Souza
• Aleluia, Milena Magalhães

Titel

Leg Ulcers in Sickle Cell Disease: A Multifactorial Analysis Highlights the Hemolytic Profile

Ist Teil von

• Hematology reports, 2023-02, Vol.15 (1), p.119-129

Ort / Verlag

Switzerland: MDPI AG

Erscheinungsjahr

2023

Quelle

EZB Electronic Journals Library

Beschreibungen/Notizen

• Sickle cell disease (SCD) is characterized by the presence of the variant S hemoglobin (HbS). The homozygous genotype (HbSS) is sickle cell anemia (SCA), while the double heterozygous of HbS and HbC (HbSC) is defined as SC hemoglobinopathy. The pathophysiology is based on chronic hemolysis, inflammation, endothelial dysfunction, and vaso-occlusion, which results in vasculopathy and serious clinical manifestations. Sickle leg ulcers (SLUs) are cutaneous lesions around the malleoli frequent in 20% of Brazilian patients with SCD. SLUs present a variable clinical and laboratory pattern modulated by several characteristics that are not fully understood. Hence, this study aimed to investigate laboratory biomarkers and genetic and clinical parameters associated with the development of SLUs. This descriptive cross-sectional study included 69 SCD patients, 52 without SLU (SLU-) and 17 with active or previous SLU history (SLU+). The results showed a higher incidence of SLU in SCA patients and there was no observed association of α-3.7 Kb thalassemia in SLU occurrence. Alterations in NO metabolism and hemolysis were associated with clinical evolution and severity of SLU, in addition to hemolysis modulating the etiology and recurrence of SLU. Our multifactorial analyses demonstrate and extend the role of hemolysis driving the pathophysiological mechanism of SLU.