Inflammation in sickle cell disease

Author(s)

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

Affiliation

^[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

• Document type: Journal article
• Source: CLINICAL HEMORHEOLOGY AND MICROCIRCULATION; v. 68, n. 2-3, p. 263-299, 2018.
• Web of Science Citations: 13

Abstract

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)

• FAPESP's process: 14/19173-5 - Vascular inflammation: pathophysiological mechanisms of induction and pathways of cellular activation
• Grantee: Nicola Amanda Conran Zorzetto
• Support Opportunities: Regular Research Grants