title: Exposure of blood from patients with sickle cell disease to air changes the morphological, oxygen-binding, and sickling properties of sickled erythrocytes.
abstract: We collected venous blood samples from 7 steady-state patients with homozygous sickle cell disease under venous oxygen pressure without exposure to air (UnExp-blood) and compared the morphological, oxygen-binding, and sickling properties with those of SS cells in aliquots of the same venous blood samples that were oxygenated in room air or at a PO2 near 180 mmHg (Exp-blood). Results showed that (1) upon deoxygenation under nitrogen, UnExp-blood generated a significantly higher percentage of elongated reversibly sickled cells (RSCs) than did Exp-blood; (2) upon gradual oxygenation of completely deoxygenated sickled cells, RSCs in UnExp-blood converted to discocytes at a higher oxygen pressure than did those in Exp-blood; (3) the degree of hysteresis between the sickling/desickling curves of UnExp-blood was greater than that of Exp-blood; and (4) deoxy-Hb S in hemolysate prepared from SS cells in UnExp-blood polymerized without a delay time, while those from Exp-blood polymerized with a distinct delay time. The in vivo properties of RSCs significantly changed upon oxygenation. We also found that the various properties of blood samples collected from patients with SCD by the ordinary method were similar to those of Exp-blood, probably because such blood samples are exposed to oxygen through air in the needle, syringe, and Vacutainer. Once SS cells were oxygenated, the in vivo properties of RSCs could not be recovered by partial deoxygenation to venous oxygen pressure.
authors.affiliation: Division of Hematology, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
authors.forename: Kazuo
authors.initials: K
authors.lastname: Obata
authors.forename: Julian
authors.initials: J
authors.lastname: Mattiello
authors.forename: Kenji
authors.initials: K
authors.lastname: Asakura
authors.forename: Kwaku
authors.initials: K
authors.lastname: Ohene-Frempong
authors.forename: Toshio
authors.initials: T
authors.lastname: Asakura
mesh_terms: D000755:Anemia, Sickle Cell / Q000097:blood / Q000473:pathology*; D001800:Blood Specimen Collection / Q000379:methods; D004907:Erythrocyte Deformability / Q000187:drug effects*; D004913:Erythrocytes, Abnormal / Q000378:metabolism / Q000473:pathology*; D006451:Hemoglobin, Sickle / Q000032:analysis / Q000378:metabolism; D006720:Homozygote; D006801:Humans; D010084:Oxidation-Reduction / Q000187:drug effects; D010100:Oxygen / Q000378:metabolism / Q000494:pharmacology*; D010313:Partial Pressure
chemical_list: D006451:Hemoglobin, Sickle; D010100:Oxygen