Diagnostic value of anemia, red blood cell morphology, and reticulocyte count for sickle cell disease.
OBJECTIVE
To determine the diagnostic value of anemia, RBC morphology, and reticulocyte count for differentiating patients with sickle cell trait from those with sickle cell disease, who have acute medical or surgical conditions and a positive sickle cell screen.


METHODS
Retrospective chart review.


METHODS
A midwest urban children's hospital with 220 beds and 36,000 emergency department visits per year.


METHODS
One hundred six patients with sickle cell trait and 152 patients with sickle cell hemoglobinopathies.


RESULTS
Anemia was observed significantly more often in patients with sickle cell disease compared with sickle cell trait (P less than .001) at all ages 3 months and older. However, anemia alone as a diagnostic test lacked high sensitivity and specificity in children less than 4 years old. Sensitivity approached 100% with the presence of anemia, abnormal RBC morphology, or reticulocyte count of more than 2%.


CONCLUSIONS
Absence of anemia alone does not exclude the diagnosis of sickle cell disease in children less than 4 years old. To differentiate trait from sickle cell disease, we recommend determination of not only hemoglobin adjusted for age but also of RBC morphology and reticulocyte count on all children presenting with acute medical and surgical conditions and a positive sickle cell screen.
Losek
J D
JD
Department of Pediatrics, Medical College of Wisconsin, Children's Hospital of Wisconsin, Milwaukee.
Hellmich
T R
TR
Hoffman
G M
GM
D000293:Adolescent; D000740:Anemia / Q000175:diagnosis / Q000209:etiology*; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications / Q000175:diagnosis*; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D004906:Erythrocyte Count; D004912:Erythrocytes / Q000473:pathology*; D005260:Female; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D007223:Infant; D008297:Male; D012156:Reticulocytes; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D012805:Sickle Cell Trait / Q000097:blood / Q000150:complications / Q000175:diagnosis*
D006454:Hemoglobins