Life-Threatening Infectious Complications in Sickle Cell Disease: A Concise Narrative Review

Frontiers in Pediatrics
Dominik OchocinskiNiranjan Kissoon

Abstract

Sickle cell disease (SCD) results in chronic hemolytic anemia, recurrent vascular occlusion, insidious vital organ deterioration, early mortality, and diminished quality of life. Life-threatening acute physiologic crises may occur on a background of progressive diminishing vital organ function. Sickle hemoglobin polymerizes in the deoxygenated state, resulting in erythrocyte membrane deformation, vascular occlusion, and hemolysis. Vascular occlusion and increased blood viscosity results in functional asplenia and immune deficiency in early childhood, resulting in life-long increased susceptibility to serious bacterial infections. Infection remains a main cause of overall mortality in patients with SCD in low- and middle-income countries due to increased exposure to pathogens, increased co-morbidities such as malnutrition, lower vaccination rates, and diminished access to definitive care, including antibiotics and blood. Thus, the greatest gains in preventing infection-associated mortality can be achieved by addressing these factors for SCD patients in austere environments. In contrast, in high-income countries, perinatal diagnosis of SCD, antimicrobial prophylaxis, vaccination, aggressive use of antibiotics for febrile episodes...Continue Reading

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Citations

Nov 23, 2020·Thrombosis Research·Valéria Sutana LadeiraDanyelle Romana Alves Rios
Apr 2, 2021·Journal of Blood Medicine·Christos VarelasEfthymia Vlachaki
Oct 10, 2021·Journal of Paediatrics and Child Health·Abirami Ganesh Kumar, Andreas Andreou

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