Erythrocyte density in sickle cell syndromes is associated with specific clinical manifestations and hemolysis.
Dense, dehydrated red blood cells (DRBCs) are a characteristic feature of sickle-cell disease (SCD). DRBCs play a role in the pathophysiology of SCD acute and chronic organ damage because of heightened tendency to undergo polymerization and sickling because of their higher hemoglobin S concentration. Relations between red cell density (assessed with phthalate density-distribution profile method) and several hematologic, biochemical, genetic parameters, and clinical manifestations were studied in a large cohort of homozygous patients. The percentage of DRBCs was significantly higher in patients who experienced skin ulcers, priapism, or renal dysfunction. Presence of α-thalassemia deletions was associated with fewer DRBCs. A multivariable analysis model showed DRBCs to be positively associated with hemolytic parameters such as lactate dehydrogenase and bilirubin and negatively with fetal hemoglobin. The percentage of DRBCs decreased by 34% at 6 months of hydroxycarbamide (xydroxyurea) therapy. Thus, DRBCs are associated with specific clinical manifestations and biologic markers and may be a useful addition to the biologic and clinical evaluation of patients with SCD, because they can easily be measured in a hematocrit tube.
Service de Médecine Interne, Hôpital Henri-Mondor, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Est, Créteil, France.
Pablo
P
Bartolucci
Carlo
C
Brugnara
Armando
A
Teixeira-Pinto
Serge
S
Pissard
Kamran
K
Moradkhani
Hélène
H
Jouault
Frederic
F
Galacteros
D000328:Adult; D000368:Aged; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications* / Q000473:pathology; D001663:Bilirubin / Q000378:metabolism; D015331:Cohort Studies; D004913:Erythrocytes, Abnormal / Q000473:pathology*; D005260:Female; D005319:Fetal Hemoglobin / Q000737:chemistry; D006400:Hematocrit; D006451:Hemoglobin, Sickle / Q000737:chemistry; D006461:Hemolysis; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D011317:Priapism / Q000209:etiology / Q000473:pathology*; D051437:Renal Insufficiency / Q000209:etiology / Q000473:pathology*; D012883:Skin Ulcer / Q000209:etiology / Q000473:pathology*; D055815:Young Adult; D017085:alpha-Thalassemia / Q000209:etiology / Q000473:pathology*
D006451:Hemoglobin, Sickle; D005319:Fetal Hemoglobin; D007770:L-Lactate Dehydrogenase; D001663:Bilirubin