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ANEMIA IN CHILDREN AND YOUNG ADULTS WITH HEMOPHILIA

Mild reductions in WBC and platelet counts are extremely common in multi-transfused hemophiliacs. Little attention has previously been directed to parameters of erythropoiesis in hemophiliacs even though anemia might be expected as a result of internal and occult external hemorrhage and impaired iron reutilization. Therefore, we examined hemoglobin (Hgb) concentration and red blood cell indices in 94 patients at times of comprehensive clinic visits. Additional studies performed in many included free erythrocyte protoporphyrin (FEP) and serum transferrin saturation, ferritin and haptoglobin. Hgb and MCV values were recorded as age-related percentile values. Hemophiliacs of all ages and degrees of severity often had lower than average values for Hgb; 31% had Hgb less than the 3rd percentile, 46% below the 10th percentile, and 83% below the mean value. MCV was less than the 3rd percentile in 11% of cases. Reduced Hgb percentile value was unrelated to age, severity of disease, or human immunodeficiency virus antibody status. Only 5 patients had an obvious cause of anemia (thalas-semia trait, blood loss, or iron deficiency). Serum ferritin, transferrin saturation, and FEP values were abnormal in just 6%, 4%, and 10% of patients respectively, indicating that iron deficiency or anemia of chronic disease was uncommon. Haptoglobin was low in 44% of patients, but reticulocyte count was > 2.5% in only 11%. In conclusion, Hgb values are commonly below the mean normal values for age in hemophiliacs. Frank anemia is present in 31% of patients, is usually mild, and is without obvious cause.