title: The red cell distribution width in sickle cell disease--is it of clinical value?
abstract: The red cell distribution width (RDW) has been studied during the clinical steady state in 1121 patients with homozygous sickle cell (SS) disease, 344 with sickle cell-haemoglobin C (SC) disease, 68 with sickle cell-beta+ thalassaemia, 49 with sickle cell beta 0 thalassaemia and in 130 control subjects with a normal (AA) genotype. The mean RDW was moderately increased in S beta + thalassaemia and SC disease and markedly increased in S beta 0 thalassaemia and SS disease. In SS, SC and S beta 0 thalassaemia genotypes, lower RDW values occurred in females and with alpha thalassaemia. The RDW correlated negatively with total haemoglobin, mean cell haemoglobin concentration, mean cell volume, and fetal haemoglobin (HbF) and positively with reticulocyte count in SS disease. A low RDW was associated with higher weight and less frequent dactylitis, painful crisis, acute chest syndrome, acute splenic sequestration, and hospital admissions. A low RDW in SS disease is consistent with a high total haemoglobin, high HbF, low reticulocyte count, alpha thalassaemia, and a more mild clinical course.
authors.affiliation: Department of Child Health, University of the West Indies, Mona, Kingston Jamaica.
authors.forename: M
authors.initials: M
authors.lastname: Thame
authors.forename: Y
authors.initials: Y
authors.lastname: Grandison
authors.forename: K
authors.initials: K
authors.lastname: Mason
authors.forename: M
authors.initials: M
authors.lastname: Thompson
authors.forename: D
authors.initials: D
authors.lastname: Higgs
authors.forename: J
authors.initials: J
authors.lastname: Morris
authors.forename: B
authors.initials: B
authors.lastname: Serjeant
authors.forename: G
authors.initials: G
authors.lastname: Serjeant
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D004909:Erythrocyte Indices; D004913:Erythrocytes, Abnormal / Q000648:ultrastructure*; D005260:Female; D005914:Globins / Q000235:genetics; D006445:Hemoglobin C Disease / Q000097:blood / Q000150:complications; D006801:Humans; D007223:Infant; D000090463:Iron Deficiencies; D008297:Male; D008875:Middle Aged; D012720:Severity of Illness Index; D012737:Sex Factors; D012805:Sickle Cell Trait / Q000097:blood* / Q000150:complications / Q000473:pathology; D013789:Thalassemia / Q000097:blood / Q000150:complications
chemical_list: D005914:Globins