Related Experiment Videos
[Infection and sickle cell anemia]
1Service de Pédiatrie, Médecine et Maladies Infectieuses, Hôpital Trousseau, Paris, France.
Insights
Sickle cell disease patients are prone to severe infections. Prophylactic penicillin and vaccinations significantly reduce early mortality and infections like pneumococcal disease.
Area of Science:
- Hematology
- Infectious Diseases
- Immunology
Context:
- Sickle cell disease (SCD) impairs immune function and causes functional asplenia, increasing infection risk.
- Infections in SCD can precipitate severe complications, including vasoocclusive crises and acute chest syndrome.
- Specific pathogens like Streptococcus pneumoniae and Salmonella pose significant threats.
Purpose:
- To review the spectrum of infections in sickle cell disease.
- To outline current prophylactic and therapeutic strategies for infection management.
- To emphasize the importance of early and continuous preventive measures.
Summary:
- SCD patients face high risks of bacterial infections (pneumonia, bacteremia, meningitis) and other serious conditions like osteomyelitis and parvovirus B19.
- Prophylactic penicillin therapy initiated at four months and comprehensive immunizations are crucial for reducing mortality.
- Antimicrobials should target common pathogens, and conjugated pneumococcal vaccines are highly effective in infants.
Impact:
- Effective infection prevention dramatically decreases early mortality in sickle cell disease.
- Long-term penicillin prophylaxis and vaccination are essential components of SCD management.
- Understanding infection risks guides optimal patient care and improves long-term outcomes.
Abstract:
Sickle cell disease 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. Pneumococcal bacteremia and meningitis are so severe as to warrant prophylactic penicillin therapy, 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, Haemophilus influenzae, Mycoplasma pneumoniae, and Chlamydia pneumoniae. Acute chest syndrome is both a difficult differential diagnosis and a common concomitant of bacterial pneumonia. Osteomyelitis is generally due to a salmonella, most often S. enteritidis; multiple foci are common and treatment is difficult, with some patients developing chronic osteomyelitis with sequestration. Parvovirus B 19 infection causes acute bone marrow failure. Malaria does not result in cerebral malaria but can lead to severe anemia or vasoocclusive crisis, and should therefore be effectively prevented. Antimicrobials are generally selected for efficacy against pneumococci (septicemia, meningitis), Salmonella (septicemia, meningitis, osteomyelitis), and mycoplasmas (pneumonia). Prophylactic therapy is of paramount importance and relies on long-term or lifelong penicillin therapy started at four months of age and on closely-spaced immunizations, most notably against pneumococci, the hepatitis B virus, S. typhi, and H. influenzae. Resistant pneumococcal strains have not been reported to cause prophylactic treatment failures. Conjugated pneumococcal vaccines are effective in protecting infants and should therefore be used in sickle cell patients.