Pitted red cell counts in sickle cell disease. Relationship to age, hemoglobin genotype, and splenic size.
Splenic reticuloendothelial function, as determined by pitted red cell counts in 114 pediatric patients with sickle cell disease, was evaluated. Patients with homozygous sickle cell disease (HbSS) had a mean pit count of 11.8 +/- 7.0% and the count increased with age. Sickle cell hemoglobin C disease (HbSC) patients had a mean pit count of 4.9 +/- 9.1%, the count being unaffected by age. One patient with HbS-beta thalassemia had a count of 12%, while eight patients with HbS-beta + thalassemia had a mean count of 0.4 +/- 0.3%. There was one patient each with HbSS-alpha thalassemia, HbS-O Arab, and HbS-Lepore, and the pit counts were 4.9, 31.4, and 0.4%, respectively. In both HbSS and HbSC patients, the pit count was significantly lower in patients who had palpable spleens. The pit count may be used as a predictor of disease severity in the sickle cell disorders.
D000293:Adolescent; D000367:Age Factors; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D004913:Erythrocytes, Abnormal / Q000473:pathology; D006450:Hemoglobin SC Disease / Q000097:blood / Q000473:pathology; D006455:Hemoglobins, Abnormal / Q000502:physiology; D006801:Humans; D007223:Infant; D013154:Spleen / Q000473:pathology; D013789:Thalassemia / Q000097:blood
D006455:Hemoglobins, Abnormal