Asian‐type DEL (RHD*DEL1) with an allo‐anti‐D: A paradoxical observation in a healthy multiparous woman

Abstract

BackgroundThe DEL phenotype is the D variant expressing the least amounts of D antigen per red cell. Asian‐type DEL (RHD:c:1227G > A) is the most prevalent DEL in East Asia without any anti‐D alloimmunization reported before. We investigated the first observation of an anti‐D in any DEL phenotype, reported in the Japanese language at a 1987 conference, only 3 years after the discovery of DEL.MethodsWe contacted the proband 35 years after the initial report. Standard hemagglutination, adsorption/elution, and flow cytometry tests were performed, as was nucleotide sequencing for the RHD, RHCE, and HLA class I and class II genes.ResultsThe healthy multiparous Japanese woman, a regular blood donor, still had the anti‐D of titer 8 representing an alloantibody by standard serologic methods. Unexpectedly, she carried an Asian‐type DEL without any additional RHD gene variation. All 12 HLA alleles identified were known in the Japanese population. Interestingly, one of her HLA‐DRB1 and a variant of her HLA‐DQB1 alleles had previously been associated with anti‐D immunization.ConclusionWe described an allo‐anti‐D, maintained for more than three decades, in an Asian‐type DEL. The combination of two implicated HLA alleles were rare and could have contributed to the anti‐D immunization. Continued monitoring of anti‐D immunization events in patients with DEL is warranted, and we discuss possible mechanisms for further study. As only this single observation has been recognized in the last 35 years, the current recommendation is affirmed: Individuals with Asian‐type DEL should be treated as Rh D‐positive for transfusion and Rh immune prophylaxis purposes.

Guide to Rho(D) Immune Globulin in Women With Molecularly Defined Asian-type DEL (c.1227G>A)

“…Alloanti-D development in transfusion recipients who received red cells from Asian-type DEL individuals has been reported in many countries [ 9 , 11 - 16 ]. There is only one report of anti-D immunization in a multiparous Japanese woman with Asian-type DEL who received D-positive red cells; however, this does not undermine the claim that individuals with Asian-type DEL are not at risk of developing alloanti-D, as the HLA-DRB1 and - DQB1 alleles she carried are associated with anti-D alloimmunization [ 17 ].…”