Infection in sickle cell disease: A review

Abstract

Infection is a significant contributor to morbidity and mortality in sickle cell disease (SCD). The sickle gene confers an increased susceptibility to infection, especially to certain bacterial pathogens, and at the same time infection provokes a cascade of SCD-specific pathophysiological changes. Historically, infection is a major cause of mortality in SCD, particularly in children, and it was implicated in 20-50% of deaths in prospective cohort studies over the last 20 years. Worldwide, it remains the leading cause of death, particularly in less developed nations. In developed countries, measures to prevent and effectively treat infection have made a substantial contribution to improvements in survival and quality of life, and are continually being developed and extended. However, progress continues to lag in less developed countries where the patterns of morbidity and mortality are less well defined and implementation of preventive care is poor. This review provides an overview of how SCD increases susceptibility to infections, the underlying mechanisms for susceptibility to specific pathogens, and how infection modifies the outcome of SCD. It also highlights the challenges in reducing the global burden of mortality in SCD.

Extracellular DNA traps promote thrombosis

“…RBC adhesion to NETs could also play a role in sickle-cell disease, where a lethal crisis is often precipitated by infection (38).…”

Human genetic basis of interindividual variability in the course of infection

“…By 1949, even before the first clinical description of the primary immunodeficiencies, sickle cell disease was known to be an autosomal recessive disorder involving hemoglobin (Hb), i.e., a molecular disease (131). It is no coincidence that immunologists, hematologists, and pulmonologists still refuse to see sickle cell disease and cystic fibrosis as primary immunodeficiencies, even though most patients with either condition die from infection (132,133). This refusal again reflects the evolution of this field since the 1950s.…”

Jaw Osteomyelitis as a Complication of Sickle Cell Anaemia in Three Omani Patients: Case reports and literature review

“…16 subsequently underwent a sequestrectomy of the right mandible with removal of teeth 45 and 46 under general anaesthesia. As tooth 47 was deemed salvageable, a root canal treatment was also performed.…”

“…During a VOC episode, the entanglement of sickled cells within the inferior alveolar artery causes blockage, infarction and pulpal necrosis. 16 These ischaemic areas may become necrotic and infected secondarily via either the haematogenous route or by local invasion through the periodontal ligaments. 16 Among reported cases of SCJO, the mandible is the most commonly involved part of the jaw, with the lower molar region being the most common mandibular site.…”