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Publications

Journal Article Research Support, N.I.H., Extramural Ppublish 3 citations

PubMed: 39003570 DOI: 10.1111/trf.17924

Chronic automated red cell exchange therapy for sickle cell disease.

Transfusion 64(8):1509-1519

Authors: Abdulhafiz Zakieh, Nicolas Mercure-Corriveau, Sophie Lanzkron, Xinyi Feng, Sonja Vozniak, Elizabeth P Crowe, Herleen Rai, Courtney Lawrence, Denise Bekkouri, Ruchika Goel, Aaron A R Tobian, Evan M Bloch

Affiliations

  • Department of Pediatrics, Division of Pediatric Hematology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. Pediatric Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA.
  • Department of Pathology, Division of Transfusion Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
  • Department of Hematology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
  • Department of Pediatrics, Division of Pediatric Hematology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
  • Vitalant, Scottsdale, Arizona, USA.

Abstract

BACKGROUND

The data to support chronic automated red cell exchange (RCE) in sickle cell disease (SCD) outside of stroke prevention, is limited, especially in adults.

STUDY DESIGN AND METHODS

A retrospective analysis was conducted of patients with SCD who were referred for chronic RCE at our institution over a 10-year period. Data that were evaluated included patient demographics, referral indications, and procedural details (e.g., vascular access, adverse events, etc.). In a subanalysis, the number of annual acute care encounters during 3 years of chronic RCE was compared with that in the year preceding the first RCE.

RESULTS

A total of 164 patients were referred for chronic RCE: median age was 28 years (interquartile range [IQR] = 22-36) at referral and 60% were female. Seventy (42.6%) were naïve to chronic transfusion (simple or RCE) prior to referral. The leading indications for referral were refractory pain (73/164, 44.5%) and iron overload (57/164, 34.7%). A total of 5090 procedures occurred during the study period (median = 19, IQR = 5-45). Of the 138 patients who had central vascular access, 8 (6%) and 16 (12%) had ≥1 central-line-related thrombosis and/or infection, respectively. Of those who were not RBC alloimmunized at initiation of RCE, 12/105 (11.4%) developed new antibodies during chronic RCE. In those 30 patients who were adherent to therapy for 3 years, there was no significant difference in acute care encounters following initiation of RCE.

CONCLUSION

Prospective clinical trials are needed to determine which patients are most likely to benefit from chronic RCE and refine selection accordingly.

MeSH

Cells, Blood Transfusion, Hematology, Anemia, Sickle Cell, Blood Component Removal, Therapeutics, Hemoglobinopathies