Lawicki Shaun, Coberly Emily A, Lee Laura A, Johnson Mary, Eichbaum Quentin
Department of Pathology, University of Hawaii, Honolulu, Hawaii.
Department of Pathology, Microbiology, and Immunology, Vanderbilt University Medical Center, Nashville, Tennessee.
Transfusion. 2018 May;58(5):1157-1162. doi: 10.1111/trf.14548. Epub 2018 Feb 25.
The Kidd-null phenotype, Jk(a-b-), occurs in individuals who do not express the JK glycoprotein. Jk(a-b-) individuals can make an antibody against the Jk3 antigen, a high-incidence antigen present in more than 99.9% of most populations. This presents many challenges to the blood bank including identification of the antibody, masking of other antibodies, and how to provide transfusion support given the rarity of Jk3-negative blood products. Kidd antibodies may cause acute and delayed hemolytic reactions as well as hemolytic disease of the fetus and newborn (HDFN). In this article, we present a series of four practical cases of pregnant women with the anti-Jk3 alloantibody that demonstrate a range of clinical presentations of Kidd-related HDFN.
We retrospectively reviewed the clinical and blood bank records for four patients and their newborns encountered at institutions in Tennessee, Missouri, Hawaii, and Guam with an anti-Jk3 identified during pregnancy.
Two cases showed no significant evidence for HDFN, while two cases were of mild-to-moderate severity requiring early delivery due to elevated middle cerebral artery (MCA) flow velocities but requiring only phototherapy for hyperbilirubinemia. No intrauterine or neonatal transfusions were necessary. Anti-Jk3 alloantibody titers ranged from 2 to 128.
Clinical manifestations of anti-Jk3 HDFN are generally mild to moderate. Anti-Jk3 titers were not found to correlate directly with HDFN severity. We suggest a titer of 16 to 32 as a cutoff for implementing enhanced monitoring of fetal MCA flow velocities, as such titers may be indicative of elevated HDFN risk.
基德血型无效表型Jk(a-b-)出现在不表达JK糖蛋白的个体中。Jk(a-b-)个体可产生针对Jk3抗原的抗体,Jk3抗原是一种高频率抗原,在大多数人群中出现率超过99.9%。这给血库带来了诸多挑战,包括抗体鉴定、其他抗体的掩盖,以及鉴于Jk3阴性血液制品的稀缺如何提供输血支持。基德抗体可能引发急性和迟发性溶血性反应以及胎儿和新生儿溶血病(HDFN)。在本文中,我们呈现了一系列4例怀有抗Jk3同种抗体的孕妇的实际病例,展示了与基德相关的HDFN的一系列临床表现。
我们回顾性分析了田纳西州、密苏里州、夏威夷州和关岛各机构遇到的4例患者及其新生儿的临床和血库记录,这些患者在孕期被鉴定出有抗Jk3抗体。
2例未显示HDFN的显著证据,而2例为轻度至中度严重程度,由于大脑中动脉(MCA)血流速度升高需要提前分娩,但仅因高胆红素血症需要光疗。无需进行宫内或新生儿输血。抗Jk3同种抗体滴度范围为2至128。
抗Jk3 HDFN的临床表现通常为轻度至中度。未发现抗Jk3滴度与HDFN严重程度直接相关。我们建议以16至32的滴度作为实施增强胎儿MCA血流速度监测的临界值,因为这样的滴度可能表明HDFN风险升高。