Department of Anesthesiology and Intensive Care, Prof. Dr. Matei Balş National Institute of Infectious Diseases, Bucharest, Romania;
Rom J Morphol Embryol. 2022 Jan-Mar;63(1):229-235. doi: 10.47162/RJME.63.1.26.
Next to A and B antigens, agglutinogen D exhibits the highest immunogenicity. Following the transfusion of D-positive red blood cells (RBCs), almost 80% of D-negative recipients develop anti-D antibodies (Abs). Subsequently, anti-D immunization further promotes the synthesis of Abs towards other blood group antigens in or outside the Rh system. The D antigen is also involved in 95% of cases of hemolytic disease of the newborn. Transfusions, hemotherapy, grafts, and obstetric history (abortions, ectopic pregnancy, births) are all risk factors for Rh isoimmunization. In the case of ABO compatibility between mother and fetus, Rh-positive fetal RBCs that have reached the maternal bloodstream are not destroyed by group agglutinins, and Rh antigenic sites are not hidden by the maternal immune system. But a Rh-negative mother with a homozygous Rh-positive husband will certainly have a Rh-positive fetus. As it has an irreversible evolution, the Rh isoimmunization once installed cannot be influenced in the sense of decreasing the Ab titer, therefore, injectable globulin has no effect. A particular case was that of a newborn with Rh system incompatibility associated with hereditary spherocytosis The clinical balance at birth reflects the severe jaundice of the female newborn of 3140 g, gestational age 38∕39 weeks, extracted by lower-segment transverse Caesarean section, with a double loop nuchal cord, Apgar score 8. Because the jaundice was severe and atypical (face and upper chest), we considered the possibility of coexistence of hemolytic disease of the newborn by Rh blood group incompatibility associated with hereditary spherocytosis, as it turned out to be true and mentioned. Changes in genes encoding proteins in the structure of the RBC membrane have amplified hemolysis induced by maternal-fetal isoimmunization in the Rh system. Massive hemolysis accentuated by congenital spherocytosis, confirmed later, imposed blood transfusion and dynamic monitoring.
除了 A 和 B 抗原外，D 抗原表现出最高的免疫原性。在输注 D 阳性红细胞（RBC）后，近 80%的 D 阴性受者会产生抗-D 抗体（Abs）。随后，抗-D 免疫进一步促进了 Rh 系统内外其他血型抗原的 Abs 合成。D 抗原也与新生儿溶血病的 95%病例有关。输血、血液疗法、移植和产科史（流产、异位妊娠、分娩）都是 Rh 同种免疫的危险因素。在母亲和胎儿 ABO 相容的情况下，已经到达母亲血流的 Rh 阳性胎儿 RBC 不会被群体凝集素破坏，Rh 抗原部位也不会被母体免疫系统隐藏。但是，Rh 阴性母亲与纯合 Rh 阳性丈夫肯定会有 Rh 阳性胎儿。由于其具有不可逆转的演变，一旦安装了 Rh 同种免疫，就无法在降低 Ab 滴度的意义上产生影响，因此，注射球蛋白没有效果。一个特殊的病例是与遗传性球形红细胞增多症相关的 Rh 系统不相容的新生儿。出生时的临床平衡反映了 3140 克的女性新生儿的严重黄疸，妊娠年龄为 38∕39 周，通过下段横向剖宫产术提取，具有双环颈索，Apgar 评分为 8。由于黄疸严重且不典型（面部和上胸部），我们考虑了与遗传性球形红细胞增多症相关的 Rh 血型不相容性引起的新生儿溶血病共存的可能性，事实证明确实如此。编码 RBC 膜结构中蛋白质的基因突变放大了母体-胎儿同种免疫在 Rh 系统中引起的溶血。随后证实的先天性球形红细胞增多症引起的大量溶血需要输血和动态监测。
