Lorch et al reported risk factors associated with excess mortality for adults with sickle cell disease (SCD). These can help to identify a patient who may benefit from more aggressive management. The authors are from Montefiore Medical Center and Albert Einstein College of Medicine in New York City.


Independent predictors of mortality identified on multivariate analysis:

(1) estimated pulmonary arterial systolic pressure (ePASP) >= 40 mm Hg at any time (hazard ratio 7)

(2) estimated glomerular filtration rate (GFR) < 60 mL per min per 1.73 square meters BSA (hazard ratio 16)

(3) total serum bilirubin > 5 mg/dL (hazard ratio 9)



• Pulmonary hypertension in SCD is discussed in 08.14.13.

• Estimating the pulmonary arterial systolic pressure from the tricuspid regurgitant jet velocity is discussed in 07.20.14.

• The elevated total serum bilirubin may reflect hemolysis and/or sickle cell hepatopathy.


A higher level of hemoglobin F may be marginally protective (hazard ratio 0.8).


The elevated pulmonary arterial systolic pressure and decreased GFR reflect the vasculopathy associated with sickle cell disease.


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