Description

Brousse et al reported markers of disease severity in infants with sickle cell anemia. These can identify a patient who may benefit from more aggressive management. The authors are from multiple institutions in France, London and New York City.


Patient selection: infant with hemoglobin SS or hemoglobin S-beta thalassemia (beta-0)

 

Outcome: severe clinical event (acute splenic sequestration, vaso-occlusive event, transfusion requirement, abnormal cerebral Doppler velocity, death)

 

Markers of a severe clinical event at enrollment:

(1) hemoglobin <= 8 g/dL

(2) percent hemoglobin F <= 33.5%

 

Hemoglobin F percentage decreased over time with mean level of 21% at 24 months.


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