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Infection in Sickle Cell Disease: a Review

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Infection in Sickle Cell Disease: a Review International Journal of Infectious Diseases (2010) 14, e2—e12 http://intl.elsevierhealth.com/journals/ijid REVIEW Infection in sickle cell disease: A review Catherine Booth a, Baba Inusa b, Stephen K. Obaro c,* a Guy’s, King’s & St Thomas’ Medical School, King’s College London, London, UK b Sickle cell and Thalassaemia Centre, Evelina Children’s Hospital, Guy’s and St Thomas’ NHS Foundation Trust, London, UK c Division of Pediatric Infectious Diseases, Department of Pediatrics and Human Development, Michigan State University, East Lansing, MI 48828, USA Received 12 November 2008; received in revised form 9 March 2009; accepted 11 March 2009 Corresponding Editor: William Cameron, Ottawa, Canada KEYWORDS Summary Infection is a significant contributor to morbidity and mortality in sickle cell disease Sickle cell disease; (SCD). The sickle gene confers an increased susceptibility to infection, especially to certain Infection; bacterial pathogens, and at the same time infection provokes a cascade of SCD-specific Hemoglobin; pathophysiological changes. Historically, infection is a major cause of mortality in SCD, parti- Spleen; cularly in children, and it was implicated in 20—50% of deaths in prospective cohort studies over Bacteria; the last 20 years. Worldwide, it remains the leading cause of death, particularly in less developed Virus 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. # 2009 International Society for Infectious Diseases. Published by Elsevier Ltd. All rights reserved. Introduction Caribbean descent, and also those from the Mediterranean, Middle East, and parts of India.2 The underlying abnormality Sickle cell disease (SCD) is a collective term for a number of is a single nucleotide substitution (GTG for GAG) in the gene genetic disorders in which hemoglobin is structurally abnor- for b-globin on chromosome 11, resulting in the replacement of a glutamic acid residue with valine on the surface of the mal, resulting in the episodic formation of sickle-shaped red 3 blood cells (RBCs) and a wide range of clinical manifesta- protein (termed HbS). In normal adult HbA, two chains of tions. It affects some 12 500 people in the UK and millions a-globin and two of b-globin form a tetramer, stabilized by worldwide,1 particularly those of black African and Afro- specific intramolecular points of contact, but without inter- actions between individual tetramers within the RBC.4 When the molecule binds or releases oxygen, it undergoes a con- * Corresponding author. formational change. In HbS, deoxygenation exposes the E-mail address: [email protected] (S.K. Obaro). abnormal valine residue on the surface of the molecule, 1201-9712/$36.00 # 2009 International Society for Infectious Diseases. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.ijid.2009.03.010 Infection in sickle cell disease e3 which then forms hydrophobic interactions with adjacent contains collections of B and T lymphocytes in follicles and chains. The resulting polymers align into bundles, causing periarteriolar lymphatic sheaths. Activation of these cells by distortion of the RBC into a crescent or sickle shape and filtered antigenic material enables initiation and expansion reducing flexibility and deformability, which impairs passage of a specific acquired immune response. Blood then enters of the cells through narrow blood vessels.3 Sickling can be the splenic cords of the red pulp, where cells flow over a fine precipitated by environmental factors such as hypoxia, low reticular meshwork and pass through fenestrated epithelium pH, cold, and dehydration of the RBC, as well as adhesion to enter the venous sinuses. This creates a slow flow, enabling molecules and cytokines associated with infections. splenic macrophages to remove defective RBCs and bacteria Homozygous SS (sickle cell anemia) is generally consid- and to present antigen to lymphocytes.10 Some bacteria can ered the most severe form of SCD. Compound heterozygotes, be recognized directly by macrophages, but many first in whom HbS is combined with a different mutation in the require opsonization—coating of the microbial surface by second b-globin gene, such as HbC, D, OArab or b-thalassemia complement components (especially C3b) or other mole- (where b-globin synthesis is reduced) can also be affected, cules, which in turn interact with receptors on phagocytes.11 with variable phenotypes. The carrier state (HbAS) does not The spleen is the site of synthesis of tuftsin, an immunosti- cause clinically significant disease (though sickling may occur mulatory peptide, and properdin, which participates in com- under extreme conditions), so carriers are most often una- plement activation.12 Opsonized bacteria are removed ware of their genotype or their sickle gene status, and the efficiently by macrophages in the spleen or liver, but poorly frequency of the gene in some populations is very high: one in opsonized bacteria are only cleared effectively by the four among Nigerians.5 spleen. Such pathogens include encapsulated bacteria, in The clinical manifestations of SCD result from two key particular Streptococcus pneumoniae (pneumococcus) and pathological processes: vaso-occlusion and hemolysis. Sickle Haemophilus influenzae. Their polysaccharide capsule cells, along with non-sickled RBCs, leukocytes, and platelets, impedes binding of complement or prevents complement form heterocellular aggregates, which adhere to the vascular assembled on the cell wall from interacting with macrophage endothelium, causing obstruction of the lumen of small blood receptors.10 Clearance of these bacteria requires anti-poly- vessels. This microcirculatory occlusion leads to acute and saccharide IgM antibodies, which facilitate phagocytosis chronic tissue ischemia and infarction, with multisystem either directly or via deposition of complement over the effects, particularly in bone, lungs, brain, kidneys, and capsule itself. A unique B cell population—IgM memory B spleen. It is responsible for acute painful episodes and crises cells—resides in the marginal zone of the spleen (adjacent to and many of the long-term complications seen in SCD. Sickled the follicles). These cells persist after an initial infection and RBCs are more readily destroyed by the reticulo-endothelial rapidly produce antibody on subsequent exposures.13 system, partly as their rigidity makes them more easily Individuals with SCD typically suffer from functional hypo- filtered in the spleen and partly due to changes in the or asplenism. The sluggish circulation through the spleen, structure of the lipid bilayer (with exposure of anionic phos- high rates of O2 extraction, and local acidosis cause deox- phatidylserine on the RBC surface), which promotes phago- ygenation of HbS, promoting sickling, which leads to conges- cytosis.3 With sickle cell anemia (HbSS), this causes a chronic tion and engorgement of the sinusoids with sickled cells. This anemia (a steady state Hb of 6—8 g/dl)6 with a resultant can cause diversion of blood via intrasplenic shunts, bypass- increase in cardiac output and workload, which produces ing the normal filtering mechanisms. Macrophages engulfing cardiomegaly and reduced exercise tolerance. The increased the abnormally shaped cells may become ‘blocked’, impair- energy demands due to this and the chronically elevated rate ing their phagocytosis of other particles. Together these of hematopoiesis contribute towards poor growth in children, effects produce a hyposplenic state that is initially reversi- and individuals are susceptible to any factor exacerbating the ble, for example if HbS levels are lowered to less than 50% by anemia, which can precipitate circulatory failure.7 Intravas- blood transfusion, bone marrow transplant, or hydroxyurea cular hemolysis also leads to release of free hemoglobin—an treatment.12 However, over time repeated episodes of sick- important scavenger of nitric oxide (NO). Reduced levels of ling and ischemic damage with progressive sclerosis of arter- this potent vasodilator and the hyperdynamic circulation ioles lead to multiple infarcts of spleen tissue. Unable to contribute further to vascular damage and occlusion, includ- regenerate, the spleen becomes scarred and atrophied, ing within larger vessels.4 Despite progress in therapy, SCD culminating in ‘autosplenectomy’, where the organ shrinks remains a cause of significant morbidity and mortality. Life to a small remnant and the individual is rendered effectively expectancy in HbSS from a multicenter study in the USA in asplenic.14 In HbSS this sequence develops from the age of 6 1994 was estimated at 42 for men and 48 for women,8 and 95% months to 3 years.12 Hyposplenic and asplenic individuals of children survive to adulthood.9 lack IgM memory B cells, suggesting a role for the spleen in their generation or function, and hence they cannot mount a rapid specific response to encapsulated organisms. Local The effect of sickle cell
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