背景:D抗原是免疫原性最强的抗原之一,是胎儿和新生儿溶血性疾病(HDFN)的最常见原因。D-表型是一种罕见的Rh变体,其中没有RhCE抗原在红细胞表面表达。具有D-表型的个体能够响应于妊娠或输血而产生称为抗Rh17(Hr°)的罕见同种抗体,并且具有与C/c和E/e抗原反应的潜力,引起严重的溶血性输血反应(HTR)和胎儿和新生儿的溶血性疾病(HDFN)。
方法:我们遇到了一例严重的HDFN,偶然发现了具有抗Rh-17抗体的母亲的D-表型。D-表型已通过分子分型以及所有家族成员的基因分型得到证实。
结论:罕见的表型,如D-个体,特别是如果同种异体免疫,在输血需要时非常令人担忧。因此,正确识别这些个体对于将他们贡献给稀有供体库和采取适当的患者血液管理策略非常重要。
BACKGROUND: D antigen is one among the most immunogenic antigens and is the most common cause of Haemolytic Disease of Fetus and Newborn (HDFN). The D-phenotype is a rare Rh variant in which none of the
RhCE antigens are expressed on the red cell surface. Individuals having D-phenotype are capable of producing a rare alloantibody named as anti-Rh17(Hr° ) in response to pregnancy or transfusion and has the potential to react with C/c and E/e antigens causing severe haemolytic transfusion reaction (HTR) and haemolytic disease of fetus and newborn (HDFN).
METHODS: We have encountered a
case of severe HDFN with an accidental discovery of D- phenotype of the mother with anti-Rh-17 antibodies. D- phenotype has been confirmed with molecular typing along with genotyping of all family members.
CONCLUSIONS: Rare phenotypes like D- individuals especially if allo-immunised are of great concern at times of transfusion requirements. Hence, proper identification of these individuals are important to contribute them to the rare donor pool and to adopt adequate patient blood management strategies.