Tags

Type your tag names separated by a space and hit enter

Red blood cell mechanical sensitivity improves in patients with sickle cell disease undergoing chronic transfusion after prolonged, subhemolytic shear exposure.
Transfusion. 2018 12; 58(12):2788-2796.T

Abstract

BACKGROUND

Sickle cell disease (SCD) is a genetically inherited hemoglobinopathy in which deoxygenated hemoglobin S polymerizes, leading to stiff red blood cells (RBCs) and inefficient microcirculatory blood flow. Transfusion therapy acts as primary and secondary prevention of ischemic stroke in SCD. Whether blood transfusion alters the mechanical sensitivity (MS) of RBCs to prolonged subhemolytic shear stress (shear) is unknown. We hypothesized that individuals with SCD undergoing chronic blood transfusion would have improved sensitivity to shear, compared with patients not undergoing transfusion therapy.

STUDY DESIGN AND METHODS

Blood suspensions from individuals with SCD not receiving (n = 15) and receiving (n = 15) chronic simple transfusion were conditioned to shear (1, 4, 16, 32, and 64 Pa) for various durations (1, 4, 16, 32, and 64 sec), and then deformability of RBCs was immediately measured. Healthy young controls (n = 15) were included for reference. A surface mesh was interpolated using the data to determine the effect of blood transfusion on MS of RBCs.

RESULTS

There was impaired RBC deformability to prolonged supraphysiologic shear in both SCD groups; however, MS improved in transfused patients when exposed to prolonged physiologic shear. Furthermore, in the transfused patients with SCD, the threshold above which subhemolytic damage occurs was similar to controls.

CONCLUSION

We found that chronic transfusion therapy normalizes the MS threshold above which RBC subhemolytic damage occurs after prolonged shear exposure in SCD. An important and novel finding in transfused patients with SCD was the improvement in RBC deformability in response to prolonged shear exposure over the physiologic range.

Links

PMC Free PDF

Authors+Show Affiliations

Simmonds MJ
Menzies Health Institute Queensland, Griffith University, Queensland, Australia.
Suriany S
Division of Hematology, Children's Hospital Los Angeles, University of Southern California Keck School of Medicine, Los Angeles, California, USA.
Ponce D
Division of Cardiology, Children's Hospital Los Angeles, University of Southern California Keck School of Medicine, Los Angeles, California, USA.
Detterich JA
Division of Cardiology, Children's Hospital Los Angeles, University of Southern California Keck School of Medicine, Los Angeles, California, USA. Department of Physiology and Biophysics, University of Southern California Keck School of Medicine, Los Angeles, California, USA.

MeSH

AdolescentAdultAnemia, Sickle CellChildErythrocyte TransfusionErythrocytesFemaleHumansMaleShear Strength

Pub Type(s)

Clinical Trial
Journal Article
Multicenter Study
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

30325033

Citation

Simmonds, Michael J., et al. "Red Blood Cell Mechanical Sensitivity Improves in Patients With Sickle Cell Disease Undergoing Chronic Transfusion After Prolonged, Subhemolytic Shear Exposure." Transfusion, vol. 58, no. 12, 2018, pp. 2788-2796.
Simmonds MJ, Suriany S, Ponce D, et al. Red blood cell mechanical sensitivity improves in patients with sickle cell disease undergoing chronic transfusion after prolonged, subhemolytic shear exposure. Transfusion. 2018;58(12):2788-2796.
Simmonds, M. J., Suriany, S., Ponce, D., & Detterich, J. A. (2018). Red blood cell mechanical sensitivity improves in patients with sickle cell disease undergoing chronic transfusion after prolonged, subhemolytic shear exposure. Transfusion, 58(12), 2788-2796. https://doi.org/10.1111/trf.14901
Simmonds MJ, et al. Red Blood Cell Mechanical Sensitivity Improves in Patients With Sickle Cell Disease Undergoing Chronic Transfusion After Prolonged, Subhemolytic Shear Exposure. Transfusion. 2018;58(12):2788-2796. PubMed PMID: 30325033.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Red blood cell mechanical sensitivity improves in patients with sickle cell disease undergoing chronic transfusion after prolonged, subhemolytic shear exposure. AU - Simmonds,Michael J, AU - Suriany,Silvie, AU - Ponce,Derek, AU - Detterich,Jon A, Y1 - 2018/10/16/ PY - 2018/04/09/received PY - 2018/07/12/revised PY - 2018/07/13/accepted PY - 2018/10/17/pubmed PY - 2019/4/10/medline PY - 2018/10/17/entrez SP - 2788 EP - 2796 JF - Transfusion JO - Transfusion VL - 58 IS - 12 N2 - BACKGROUND: Sickle cell disease (SCD) is a genetically inherited hemoglobinopathy in which deoxygenated hemoglobin S polymerizes, leading to stiff red blood cells (RBCs) and inefficient microcirculatory blood flow. Transfusion therapy acts as primary and secondary prevention of ischemic stroke in SCD. Whether blood transfusion alters the mechanical sensitivity (MS) of RBCs to prolonged subhemolytic shear stress (shear) is unknown. We hypothesized that individuals with SCD undergoing chronic blood transfusion would have improved sensitivity to shear, compared with patients not undergoing transfusion therapy. STUDY DESIGN AND METHODS: Blood suspensions from individuals with SCD not receiving (n = 15) and receiving (n = 15) chronic simple transfusion were conditioned to shear (1, 4, 16, 32, and 64 Pa) for various durations (1, 4, 16, 32, and 64 sec), and then deformability of RBCs was immediately measured. Healthy young controls (n = 15) were included for reference. A surface mesh was interpolated using the data to determine the effect of blood transfusion on MS of RBCs. RESULTS: There was impaired RBC deformability to prolonged supraphysiologic shear in both SCD groups; however, MS improved in transfused patients when exposed to prolonged physiologic shear. Furthermore, in the transfused patients with SCD, the threshold above which subhemolytic damage occurs was similar to controls. CONCLUSION: We found that chronic transfusion therapy normalizes the MS threshold above which RBC subhemolytic damage occurs after prolonged shear exposure in SCD. An important and novel finding in transfused patients with SCD was the improvement in RBC deformability in response to prolonged shear exposure over the physiologic range. SN - 1537-2995 UR - https://www.unboundmedicine.com/medline/citation/30325033/Red_blood_cell_mechanical_sensitivity_improves_in_patients_with_sickle_cell_disease_undergoing_chronic_transfusion_after_prolonged_subhemolytic_shear_exposure_ DB - PRIME DP - Unbound Medicine ER -