Chronic transfusion therapy improves but does not normalize systemic and pulmonary vasculopathy in sickle cell disease

Author:

Detterich Jon A.12ORCID,Kato Roberta M.3,Rabai Miklos4,Meiselman Herbert J.2,Coates Thomas D.5,Wood John C.1ORCID

Affiliation:

1. Division of Cardiology, Children’s Hospital Los Angeles, University of Southern California Keck School of Medicine, Los Angeles, CA;

2. Department of Physiology and Biophysics, University of Southern California Keck School of Medicine, Los Angeles, CA;

3. Division of Pediatric Pulmonology, Children’s Hospital Los Angeles, Los Angeles, CA;

4. Department of Medicine, University of Pecs, Pecs, Hungary; and

5. Division of Hematology, Children’s Hospital Los Angeles, University of Southern California Keck School of Medicine, Los Angeles, CA

Abstract

Key PointsPlasma free hemoglobin is associated with abnormal systemic and pulmonary vascular function markers. Red cell storage time and physical changes in blood are associated with acute transfusional changes in endothelial function.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Reference44 articles.

1. A rapid diagnostic test for sickle cell anemia.;Itano;Blood,1949

2. Sickle cell anemia a molecular disease.;Pauling;Science,1949

3. Pain management of sickle cell disease.;Ballas;Hematol Oncol Clin North Am,2005

4. Pain in sickle cell disease. Rates and risk factors.;Platt;N Engl J Med,1991

5. Hemoglobin S polymerization and red cell membrane changes.;Kuypers;Hematol Oncol Clin North Am,2014

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