The diagnosis of iron deficiency anemia in sickle cell disease
E Vichinsky, K Kleman, S Embury and B Lubin
We determined the prevalence and optimal methods for laboratory diagnosis
of iron deficiency anemia in patients with sickle cell disease. Laboratory
investigations of 38 nontransfused and 32 transfused patients included
transferrin saturation, serum ferritin, mean corpuscular volume (MCV), and
free erythrocyte protoporphyrin (FEP). Response to iron supplementation
confirmed the diagnosis of iron deficiency anemia in 16% of the
nontransfused patients. None of the transfused patients were iron
deficient. All iron-deficient patients (mean age 2.4 yr) had a low MCV,
serum ferritin less than 25 ng/ml, transferrin saturation less than 15%,
and FEP less than 90 micrograms/dl RBC. Following therapy, all parameters
improved and the hemoglobin concentration increased greater than 2 g/dl. A
serum ferritin below 25 ng/ml was the most reliable screening test for iron
deficiency. There were 13% false positive results with transferrin
saturation, 3% with MCV, and 62% with FEP. FEP values correlated strongly
with reticulocyte counts. The high FEP was in part due to protoporphyrin IX
and not completely due to zinc protoporphyrin, which is elevated in iron
deficiency. We conclude that iron deficiency anemia is a potential problem
in young nontransfused sickle cell patients. Serum ferritin below 25 ng/ml
and low MCV are the most useful screening tests.
Volume 58,
Issue 5,
pp. 963-968,
11/01/1981
Copyright © 1981 by The American Society of Hematology
