Description

The Cooperative Study of Sickle Cell Disease (CSSCD) identified a number of predictors for adverse outcome in pediatric patients with sickle cell disease.


 

Risk factors for death as an adult:

(1) acute anemia

(2) low levels of hemoglobin F

(3) acute renal failure

(4) frequent painful crises

(5) acute chest syndrome

(6) leukocytosis

 

Risk factors for pediatric death:

(1) anemia

(2) low levels of hemoglobin F

(3) leukocytosis

(4) reticulocytosis

(5) stroke

 

Risk factors for stroke:

(1) acute anemia

(2) acute chest syndrome

(3) transient ischemic attack

(4) hypertension

(5) absence of alpha-thalassemia

 

Risk factors for acute chest syndrome:

(1) polycythemia

(2) low levels of hemoglobin F

(3) leukocytosis

 

Risk factors for painful crises:

(1) polycythemia

(2) low levels of hemoglobin F

 

Risk factors for avascular necrosis of bone:

(1) polycythemia

(2) frequent painful crises

(3) presence of alpha-thalassemia

(4) decreased MCV (microcytic indices)

(5) elevated AST

 

Risk factors for leg ulcers:

(1) anemia

(2) decreased hemoglobin F

 

Sepsis:

(1) increased percent pock-marked RBCs

 

where:

• A history of stroke, painful crises and acute chest syndrome should increase the risk for future recurrences.

 

Depressed levels of hemoglobin F is associated with:

(1) pediatric or adult death

(2) acute chest syndrome

(3) frequent painful crises

(4) leg ulcers

 


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