Tuson M, Hue-Roye K, Koval K, Imlay S, Desai R, Garg G, Kazem E, Stockman D, Hamilton J, Reid M E
Trinity Health, Farmington Hills, MI, USA.
Immunohematology. 2011;27(2):58-60.
Antibodies to antigens in the Kell blood group system are usually immunoglobulin G, and, notoriously, anti-K, anti-k, and anti-Kp(a) can cause severe hemolytic transfusion reactions, as well as severe hemolytic disease of the fetus and newborn (HDFN). It has been shown that the titer of anti-K does not correlate with the severity of HDFN because, in addition to immune destruction of red blood cells (RBCs), anti-K causes suppression of erythropoiesis in the fetus, which can result in severe anemia. We report a case involving anti-Kp(a) in which one twin was anemic and the other was not. Standard hemagglutination and polymerase chain reaction (PCR)-based tests were used. At delivery, anti-Kp(a) was identified in serum from the mother and twin A, and in the eluate prepared from the baby’s RBCs. PCR-based assays showed twin A (boy) was KEL841T/C (KEL03/KEL04), which is predicted to encode Kp(a+b+). Twin B (girl) was KEL841C/C (KEL04/KEL04), which is predicted to encode Kp(a–b+). We describe the first reported case of probable suppression of erythropoiesis attributable to anti-Kp(a). One twin born to a woman whose serum contained anti-Kp(a) experienced HDFN while the other did not. Based on DNA analysis, the predicted blood type of the affected twin was Kp(a+b+) and that of the unaffected twin was Kp(a–b+). The laboratory findings and clinical course of the affected twin were consistent with suppression of erythropoiesis in addition to immune RBC destruction.
凯尔血型系统中针对抗原的抗体通常为免疫球蛋白G,而且,众所周知,抗-K、抗-k和抗-Kp(a)可引起严重的溶血性输血反应,以及严重的胎儿和新生儿溶血病(HDFN)。已经表明,抗-K的滴度与HDFN的严重程度不相关,因为除了对红细胞(RBC)的免疫破坏外,抗-K还会导致胎儿红细胞生成受抑制,进而可能导致严重贫血。我们报告了一例涉及抗-Kp(a)的病例,其中一对双胞胎中一个贫血而另一个没有。使用了标准血凝试验和基于聚合酶链反应(PCR)的检测方法。分娩时,在母亲和双胞胎A的血清中以及从婴儿红细胞制备的洗脱液中发现了抗-Kp(a)。基于PCR的检测显示双胞胎A(男孩)为KEL841T/C(KEL03/KEL04),预计编码Kp(a+b+)。双胞胎B(女孩)为KEL841C/C(KEL04/KEL04),预计编码Kp(a–b+)。我们描述了首例可能归因于抗-Kp(a)的红细胞生成受抑制的报道病例。一名血清中含有抗-Kp(a)的女性所生的一对双胞胎中,一个发生了HDFN而另一个没有。基于DNA分析,受影响双胞胎的预测血型为Kp(a+b+),未受影响双胞胎的预测血型为Kp(a–b+)。受影响双胞胎的实验室检查结果和临床病程与除免疫性红细胞破坏外的红细胞生成受抑制情况相符。