Inflammation in sickle cell disease

Autor(es)

Conran, Nicola ^[1] ; Belcher, John D. ^[2]

Afiliação do(s) autor(es)

^[1] Univ Campinas UNICAMP, Hematol Ctr, Cidade Univ, Campinas, SP - Brazil

^[2] Univ Minnesota, Dept Med, Vasc Biol Ctr, Div Hematol Oncol & Transplantat, Box 736 UMHC, Minneapolis, MN 55455 - USA

• Tipo de documento: Artigo Científico
• Fonte: CLINICAL HEMORHEOLOGY AND MICROCIRCULATION; v. 68, n. 2-3, p. 263-299, 2018.
• Citações Web of Science: 13

Resumo

The primary beta-globin gene mutation that causes sickle cell disease (SCD) has significant pathophysiological consequences that result in hemolytic events and the induction of the inflammatory processes that ultimately lead to vaso-occlusion. In addition to their role in the initiation of the acute painful vaso-occlusive episodes that are characteristic of SCD, inflammatory processes are also key components of many of the complications of the disease including autosplenectomy, acute chest syndrome, pulmonary hypertension, leg ulcers, nephropathy and stroke. We, herein, discuss the events that trigger inflammation in the disease, as well as the mechanisms, inflammatory molecules and cells that propagate these inflammatory processes. Given the central role that inflammation plays in SCD pathophysiology, many of the therapeutic approaches currently under pre-clinical and clinical development for the treatment of SCD endeavor to counter aspects or specific molecules of these inflammatory processes and it is possible that, in the future, we will see anti-inflammatory drugs being used either together with, or in place of, hydroxyurea in those SCD patients for whom hematopoietic stem cell transplants and evolving gene therapies are not a viable option. (AU)

• Processo FAPESP: 14/19173-5 - Inflamação vascular: mecanismos fisiopatológicos de indução e vias de ativação celular
• Beneficiário: Nicola Amanda Conran Zorzetto
• Modalidade de apoio: Auxílio à Pesquisa - Regular