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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
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Chronic organ injuries in children with sickle cell disease

Slimane Allali1, Melissa Taylor2, Joséphine Brice3

  • 1Department of General Pediatrics and Pediatric Infectious Diseases, Reference Center for Sickle Cell Disease, Necker Hospital for Sick Children, Assistance Publique - Hôpitaux de Paris (AP-HP), Université de Paris, Paris; Laboratory of Cellular and Molecular Mechanisms of Hematological Disorders and Therapeutical Implications, Université de Paris, Imagine Institute, Inserm U1163, Paris; Laboratory of Excellence GR-Ex.

Haematologica
|February 25, 2021
PubMed

Insights

Sickle cell disease patients face premature death from organ failure despite increased life expectancy. Early screening and interventions like transcranial Doppler can prevent strokes, but other organ damage monitoring needs further research.

Area of Science:

  • Hematology
  • Cardiology
  • Nephrology
  • Neurology

Background:

  • Sickle cell disease (SCD) median life expectancy has improved to 55 years.
  • Premature death in SCD patients often results from organ failure, including pulmonary hypertension, kidney injury, and cerebral vasculopathy.
  • Organ damage in SCD can be asymptomatic and requires early, systematic screening.

Purpose of the Study:

  • To review current screening and therapeutic strategies for organ damage in sickle cell disease.
  • To identify prognostic indicators and effective interventions for SCD-related complications.
  • To highlight areas requiring further research in SCD management.

Main Methods:

  • Review of existing literature on screening protocols and therapeutic interventions for SCD organ damage.
  • Analysis of diagnostic tools such as transcranial Doppler and echocardiography.
  • Evaluation of biomarkers like albuminuria for kidney injury prediction.

Main Results:

  • Transcranial Doppler screening combined with transfusions significantly reduces stroke risk.
  • Increased albuminuria predicts kidney injury in SCD patients.
  • Prognostic value of tricuspid regurgitant jet velocity in children is undetermined; screening for hip and eye disorders is recommended but lacks standardized strategies.

Conclusions:

  • Systematic screening is crucial for early detection of asymptomatic organ damage in SCD.
  • While stroke prevention is established, consensus on screening and therapy for silent cerebral infarcts is lacking.
  • Further research is needed to confirm the benefits of hydroxyurea and evaluate new drugs for preventing chronic organ damage in SCD.

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