抗jkb引起的延迟溶血性输血反应:强调早期血库咨询重要性的病例报告及文献复习

D. M. Nguyen, H. J. Lee, D. Mirabella, D. W. Wu
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引用次数: 1

摘要

迟发性溶血性输血反应(DHTR)可无症状或模仿其他情况,并可能被误诊。未能认识到这一实体可能导致不适当的治疗和未来的输血反应。Anti-JK a和Anti-JK b是引起DHTR ac的最常见抗体,在极少数情况下伴有血管内溶血。由于抗体的短暂性和频繁的剂量效应,通常很难检测到抗体。我们报告一例40岁女性因意外的弱反应性抗jk b而患有DHTR。患者既往有高血压、多次输血、多次流产、子宫肌瘤所致阴道出血等病史,以腰痛4天、尿红棕色1天就诊于急诊科。体格检查发现明显黄疸。实验室数据显示血红蛋白(Hb)低(6.0g/dl),肌酐升高(3.11 mg/dl)。鉴别诊断包括溶血性尿毒症综合征、尿路结石引起的血尿和自身免疫性溶血性贫血。患者血样中观察到明显的溶血现象。进一步的询问引出了7天前的一次输血。当时的红细胞抗体检查显示存在抗k和抗e。患者输注2单位K、E抗原阴性交叉配型相容填充红细胞(PRBCs)。输血后Hb为8.8 g/dl,没有任何立即并发症的迹象。回顾最近的输血史和目前的发现,我们强烈怀疑是血管内溶血。病人接受了输液和静脉利尿剂的大力治疗。随后的Stat检查证实了血管内溶血。在启动抗体检查时,一个“新的”输血请求被搁置。红细胞抗体检查结果证实存在抗k和抗e;直接抗球蛋白试验(DAT)阴性。间接抗球蛋白试验表明,从患者红细胞制备的洗脱液无反应性。额外的样本被送到参比实验室进一步调查意外的红细胞抗体。参考实验室的结果证实了抗e和- k,此外,在血浆和洗脱液中发现了弱抗JK b。两种先前输入的单位随后被证明是Jk(b+),进一步证明抗Jk b很可能是DHTR的罪魁祸首。患者随后输注E-、K-和Jk(b-)红细胞,病情得到改善。该病例强调了血库咨询对DHTR的早期治疗和诊断以及避免不相容血液成分输血的核心作用,从而最大限度地减少发病率风险并降低死亡率的可能性。我们的意见是,当患者在最近输血后Hb急剧下降时,应咨询血库。输血医学应成为医学院教育和医院大会诊的一部分,提高临床医生对DHTR的认识和报告,及时诊断和治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Delayed Hemolytic Transfusion Reaction due to Anti-Jkb: Case Report Highlighting the Importance of Early Blood Bank Consultation and Literature Review
Delayed hemolytic transfusion reactions (DHTR) can be asymptomatic or mimic other conditions and may be misdiagnosed. Failure to recognize this entity could lead to inappropriate treatment and future transfusions reactions. Anti-JK a and anti-JK b are the most frequently encountered antibodies responsible for DHTR ac companied by intravascular hemolysis on rare occasions. The antibodies are often difficult to detect because of their transient nature and their frequent dosage effect. We report the case of a 40-year-old female with DHTR due to unexpected weakly reactive anti-JK b . The patient, with a medical history of hypertension, multiple transfusions, multiple abortions and vaginal bleeding due to uterine fibroids, presented to our emergency department complaining of back pain for four days and red-brown color urine for one day. Significant jaundice was noted on physical examination. Laboratory data showed low hemoglobin (Hb) (6.0g/dl) and increased creatinine (3.11 mg/dl). Differential diagnosis included hemolytic-uremic syndrome, hematuria caused by urinary tract calculi, and autoimmne hemolytic anemia. Significant hemolysis was observed in the patient’s blood sample.    Further   questioning   elicited   a   recent  blood  transfusion seven days prior. Red cell antibody work-up at that time showed the presence of anti-K and anti-E. The patient was transfused 2 units of K and E antigen negative cross-match compatible packed red blood cells(PRBCs). Post-transfusion Hb was 8.8 g/dl and there were no signs of any immediate complication. Review of the recent history of blood transfusion and the current findings led to our strong suspicion of intravascular hemolysis. The patient was treated vigorously with fluids and intravenous diuretics. Stat tests subsequently confirmed the intravascular hemolysis. A ‘new’ request for blood transfusion was put on hold while an antibody workup was initiated. Red cell antibody work-up results confirmed the presence of anti-K and anti-E; the direct antiglobulin test (DAT) was negative. An eluate prepared from the patient’s red cells was non-reactive by indirect antiglobulin test. Additional samples were sent to a reference laboratory for further investigation of unexpected red cell antibodies. Results from the reference laboratory confirmed the anti-E and -K, and, in addition, a weak anti- JK b was identified in the plasma and eluate. Both of the prior transfused units were subsequently shown to be Jk(b+), adding further evidence that the anti- JK b is most likely the culprit of the DHTR. The patient was subsequently transfused with E-, K- and Jk(b-) PRBCs and her condition improved. This case emphasizes the central role of blood bank consultation for early treatment and diagnosis of DHTR and for the avoidance of incompatible blood component transfusion, thus minimizing the risks of morbidity and reduce the potential for mortality. It is our opinion that blood bankers should be consulted when patients have an acute drop in Hb following recent transfusions. Moreover, transfusion medicine should be part of medical school education and part of hospital grand round conferences, to raise clinicians’ awareness to improve recognition and reporting of DHTR so that timely diagnosis and treatment can be made.
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