Partial C antigen in sickle cell disease patients: clinical relevance and prevention of alloimmunization.

Abstract:

BACKGROUND:Partial Rh antigens have been widely described in black individuals. Carriers are prone to immunization when exposed to the normal antigens. In sickle cell disease (SCD), patient alloimmunization is a major cause of transfusion failure. The potential of individuals with partial C antigen to make anti-C has not been investigated. We sought partial C status and anti-C production in a cohort of SCD patients with the C+ phenotype, to determine whether exposure to normal C antigen should be avoided. STUDY DESIGN AND METHODS:We constituted a cohort of 177 randomly selected SCD patients expressing C antigen. We screened for (C)ce(s) and R(N) haplotypes, presumably associated with partial C antigen in Afro-Caribbeans, and we recorded the number of transfused C+ red blood cell (RBC) units, immunization status, and extended phenotype. RESULTS:Forty-nine patients carried abnormal C antigen, deduced from the presence of (C)ce(s) and/or R(N), not compensated by a normal RHC allele in trans. Among patients with partial C phenotype exposed repeatedly to C+ RBCs, 30% produced anti-C. Two patients experienced hemolysis. In our hospital, with 22% of SCD patients expressing C, prevention of anti-C immunization for all individuals with partial C antigen would require a 7% increase in the use of C- RBC units. These RBCs are already in short supply for SCD patients who are C-. CONCLUSION:This study demonstrates the need to detect partial C within C+ SCD patients and to prevent immunization. A larger number of Afro-Caribbeans donors is needed to provide these patients with C- RBCs.

journal_name

Transfusion

journal_title

Transfusion

authors

Tournamille C,Meunier-Costes N,Costes B,Martret J,Barrault A,Gauthier P,Galactéros F,Nzouékou R,Bierling P,Noizat-Pirenne F

doi

10.1111/j.1537-2995.2009.02382.x

subject

Has Abstract

pub_date

2010-01-01 00:00:00

pages

13-9

issue

1

eissn

0041-1132

issn

1537-2995

pii

TRF2382

journal_volume

50

pub_type

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