Related Experiment Videos
[Severe infections in children with sickle cell disease: clinical aspects and prevention]
P Bégué1, B Castello-Herbreteau
1Service de pédiatrie générale, pathologie infectieuse et drépanocytose, hôpital Armand-Trousseau, 24, avenue du Dr Arnold-Netter, 75012 Paris, France. consultation.begue@trs.ap.hop.paris.fr
Insights
Sickle-cell disease patients face severe infections due to impaired immunity. Lifelong penicillin prophylaxis and vaccinations are crucial for preventing life-threatening bacterial infections like pneumococcal disease.
Area of Science:
- Hematology
- Infectious Diseases
- Immunology
Context:
- Sickle-cell disease (SCD) impairs immune function, leading to increased susceptibility to infections.
- Infections in SCD patients can trigger vasoocclusive crises and are often severe and life-threatening.
- Functional asplenia in SCD further compromises the immune system's ability to fight pathogens.
Purpose:
- To highlight the significant infection risks in sickle-cell disease patients.
- To emphasize the importance of prophylactic antimicrobial therapy and vaccinations.
- To discuss common and severe infections encountered in SCD, including pneumococcal disease, osteomyelitis, and parvovirus B19.
Summary:
- Pneumococcal infections (bacteremia, meningitis) are a major cause of mortality in young SCD patients, necessitating penicillin prophylaxis.
- Bacterial pneumonia, osteomyelitis (often Salmonella), and acute chest syndrome are common, requiring targeted antimicrobial treatment.
- Malaria and Parvovirus B19 infections pose additional risks, leading to severe anemia or erythroblastopenia.
Impact:
- Penicillin prophylaxis has dramatically reduced early mortality from bacterial pneumonia in SCD.
- Vaccinations against pneumococci, hepatitis B, S. typhi, and H. influenzae are essential for comprehensive prevention.
- New conjugated pneumococcal vaccines offer effective protection for infants and should be prioritized in SCD management.
Abstract:
Sickle-cell disease (SCD) is associated with frequent and often severe infections as a result of immune function impairment and functional asplenia. Also, infection can trigger a vasoocclusive crisis. Pneumonococcal bacteremia and meningitis due to S. pneumoniae are often lethal and justify the penicillin prophylaxis, which has provided a dramatic decrease in early mortality bacterial pneumonia is common in patients younger than four years, with most cases being due to S. pneumoniae, H. influenzae, Mycoplasma pneumoniae, Chlamydia pneumoniae. Acute chest syndrome is both a difficult differential diagnosis and a common concomitant of bacterial pneumonia, because they are often intricated. Osteomyelitis is generally due to Salmonella, most often S. enteritidis. Multiple foci are common and treatment is difficult, with some patients developing chronic osteomyelitis with sequestration. Osteomyelitis is less frequent in developed countries and must been differentiated with bone infarction by use of bone scintigraphy. Parvovirus B19 infection causes acute erythroblastopenias. Malaria does not result in cerebral malaria, but can lead to severe anaemia or vasoocclusive crisis, and should therefore be effectively prevented. Antimicrobials are generally selected for efficacy against pneumococci (septicemia, meningitis), Salmonella (osteomyelitis, meningitis), and M. pneumoniae (pneumonia). Prophylactic therapy is of paramount importance and relies on long-term or lifelong penicillin therapy started at three months of age and no closely-spaced immunizations, most notably against peumococci, hepatitis B virus, S. typhi and H. influenzae. Resistant pneumococcal strains have not been reported to cause prophylactic treatment failures. New conjugated pneumococcal vaccines are effective in protecting very young infants and should therefore be used in sickle cell patients.