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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
05:23

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload

Published on: March 14, 2017

[Stroke prevention in sickle-cell disease: results, hurdles and future perspectives]

Françoise Bernaudin1, Suzanne Verlhac

  • 1Hématologie-Pédiatrie, Hôpital Intercommunal de Créteil, 40 avenue de Verdun 94000 Créteil.

Bulletin De L'Academie Nationale De Medecine
|May 19, 2009
PubMed

Insights

Stroke is a serious complication of sickle cell disease (SCD). Transcranial Doppler (TCD) screening in infants detects cerebral vasculopathy, and early intervention significantly reduces stroke risk.

Area of Science:

  • Pediatric Neurology
  • Hematology
  • Vascular Medicine

Background:

  • Sickle cell disease (SCD) is the leading cause of stroke in infants.
  • Stroke represents the most severe complication of SCD in children.
  • Proximal vasculopathy accounts for 75% of SCD-related strokes, often progressive.

Purpose of the Study:

  • To evaluate the effectiveness of transcranial Doppler (TCD) screening for detecting cerebral vasculopathy in infants with SCD.
  • To assess the impact of early intervention, including transfusion programs, on stroke risk reduction in children with SCD.
  • To explore the role of stem cell transplantation as a curative treatment for high-risk SCD patients.

Main Methods:

  • Utilized transcranial Doppler (TCD) to screen for cerebral vasculopathy in infants with SCD.
  • Implemented transfusion programs to maintain HbS levels below 30% in at-risk children.
  • Analyzed stroke incidence in a cohort of screened SCD newborns compared to historical data.

Main Results:

  • High TCD velocities (> 2 m/s) indicate a 40% stroke risk within 36 months.
  • Transfusion programs reduced stroke risk from 11% to under 2% in screened SCD patients.
  • The implemented screening and intervention strategy reduced stroke risk to less than 2% by age 18.

Conclusions:

  • Routine TCD screening at 12-18 months is crucial for early detection of cerebral vasculopathy in children with SCD.
  • Proactive management, including transfusions, dramatically lowers stroke risk.
  • Genoidentical stem cell transplantation offers a high chance of cure and should be considered for high-risk individuals.

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