Variant RHD alleles and Rh immunization in patients with sickle cell disease

Kaoru Takasaki; David F. Friedman; Stacey Uter; Sunitha Vege; Connie M. Westhoff; Stella T. Chou

doi:10.1111/bjh.18774

journal article Apr 01, 2023

Variant RHD alleles and Rh immunization in patients with sickle cell disease

Kaoru Takasaki

David F. Friedman

Stacey Uter Sunitha Vege

Connie M. Westhoff

Stella T. Chou

British Journal of Haematology Vol. 201 No. 6 pp. 1220-1228 · Wiley

View at Publisher Save 10.1111/bjh.18774

Abstract

SummaryRH diversity among patients and donors contributes to Rh immunization despite serologic Rh‐matched red cell transfusions. Anti‐D can occur in D+ patients with RHD variants that encode partial D antigens. Anti‐D has also been reported in patients with conventional RHD transfused primarily with units from Black donors who frequently have variant RHD. We report 48 anti‐D in 690 D+ transfused individuals with sickle cell disease, categorized here as expressing conventional D, partial D or D antigen encoded by RHD*DAU0. Anti‐D formed in a greater proportion of individuals with partial D, occurred after fewer D+ unit exposures, and remained detectable for longer than for those in the other categories. Among all anti‐D, 13 had clinical or laboratory evidence of poor transfused red cell survival. Most individuals with anti‐D were chronically transfused, including 32 with conventional RHD who required an average of 62 D− units/year following anti‐D. Our findings suggest that patients with partial D may benefit from prophylactic D− or RH genotype‐matched transfusions to prevent anti‐D. Future studies should investigate whether RH genotype‐matched transfusions can improve use of valuable donations from Black donors, reduce D immunization and minimize transfusion of D− units to D+ individuals with conventional RHD or DAU0 alleles.

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References

Details

Published: Apr 01, 2023
Vol/Issue: 201(6)
Pages: 1220-1228
License: View

Authors

K

Kaoru Takasaki

Division of Hematology, Department of Pediatrics The Children's Hospital of Philadelphia Philadelphia Pennsylvania 19104 USA

D

David F. Friedman

Division of Hematology, Department of Pediatrics The Children's Hospital of Philadelphia Philadelphia Pennsylvania 19104 USA; Division of Transfusion Medicine, Department of Pathology and Laboratory Medicine Perelman School of Medicine at the University of Pennsylvania Philadelphia Pennsylvania 19104 USA

S

Stacey Uter

Division of Hematology, Department of Pediatrics The Children's Hospital of Philadelphia Philadelphia Pennsylvania 19104 USA

S

Sunitha Vege

Immunohematology and Genomics New York Blood Center New York New York USA

C

Connie M. Westhoff

Immunohematology and Genomics New York Blood Center New York New York USA

S

Stella T. Chou

Division of Hematology, Department of Pediatrics The Children's Hospital of Philadelphia Philadelphia Pennsylvania 19104 USA; Division of Transfusion Medicine, Department of Pathology and Laboratory Medicine Perelman School of Medicine at the University of Pennsylvania Philadelphia Pennsylvania 19104 USA

Funding

National Heart, Lung, and Blood Institute Award: R01HL147879‐01

American Society of Hematology

Cite This Article

Kaoru Takasaki, David F. Friedman, Stacey Uter, et al. (2023). Variant RHD alleles and Rh immunization in patients with sickle cell disease. British Journal of Haematology, 201(6), 1220-1228. https://doi.org/10.1111/bjh.18774

Variant RHD alleles and Rh immunization in patients with sickle cell disease

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