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Inflammation in sickle cell disease.
Clin Hemorheol Microcirc 2018; 68(2-3):263-299CH

Abstract

The primary β-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.

Authors+Show Affiliations

Hematology Center, University of Campinas - UNICAMP, Cidade Universitária, Campinas-SP, Brazil.Department of Medicine, Division of Hematology, Oncology and Transplantation, Vascular Biology Center, University of Minnesota, Minneapolis, MN, USA.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

29614637

Citation

Conran, Nicola, and John D. Belcher. "Inflammation in Sickle Cell Disease." Clinical Hemorheology and Microcirculation, vol. 68, no. 2-3, 2018, pp. 263-299.
Conran N, Belcher JD. Inflammation in sickle cell disease. Clin Hemorheol Microcirc. 2018;68(2-3):263-299.
Conran, N., & Belcher, J. D. (2018). Inflammation in sickle cell disease. Clinical Hemorheology and Microcirculation, 68(2-3), pp. 263-299. doi:10.3233/CH-189012.
Conran N, Belcher JD. Inflammation in Sickle Cell Disease. Clin Hemorheol Microcirc. 2018;68(2-3):263-299. PubMed PMID: 29614637.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Inflammation in sickle cell disease. AU - Conran,Nicola, AU - Belcher,John D, PY - 2018/4/5/entrez PY - 2018/4/5/pubmed PY - 2018/5/31/medline KW - Cytokine KW - endothelium KW - hemolysis KW - hydroxyurea KW - vaso-occlusion SP - 263 EP - 299 JF - Clinical hemorheology and microcirculation JO - Clin. Hemorheol. Microcirc. VL - 68 IS - 2-3 N2 - The primary β-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. SN - 1875-8622 UR - https://www.unboundmedicine.com/medline/citation/29614637/Inflammation_in_sickle_cell_disease L2 - https://content.iospress.com/openurl?genre=article&id=doi:10.3233/CH-189012 DB - PRIME DP - Unbound Medicine ER -