Gniadek Thomas J, McGonigle Andrea M, Shirey R Sue, Brunker Patricia A, Streiff Michael, Philosophe Benjamin, Bloch Evan M, Ness Paul M, King Karen E
Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, Minnesota.
Department of Pathology and Laboratory Medicine, Wing-Kwai and Alice Lee-Tsing Chung Transfusion Service, David Geffen School of Medicine at the University of California-Los Angeles, Los Angeles, California.
Transfusion. 2017 May;57(5):1262-1266. doi: 10.1111/trf.14055. Epub 2017 Mar 28.
Passenger lymphocyte syndrome occurs when donor lymphocytes are transplanted with a solid organ and produce alloantibodies that react with antigens on the recipient's red blood cells (RBCs). Typically, passenger lymphocyte syndrome presents as immunoglobulin G antibody-mediated, extravascular hemolytic anemia with reticulocytosis. Often, the donor was alloimmunized before transplantation.
A 34-year-old Group O, D+ man with a negative antibody screen received a liver transplant from a Group O, D- donor. Twenty Group O, D+ RBC units were transfused on Postoperative Days (PODs) 0 through 2. On POD 7, the patient developed anemia, a weakly positive antibody screen, and a positive direct antiglobulin test with anti-D in the eluate. After POD 8, a D- transfusion protocol was initiated. Despite laboratory evidence of hemolysis, two initial peripheral blood smears showed no increase in schistocytes or spherocytes, the reticulocyte count was depressed, and a marrow biopsy revealed erythroid hyperplasia. Eventually, anemia resolved after a period of medication non-compliance; however, a positive direct antiglobulin test persisted to the last follow-up date (POD 233).
Other potential causes of aplastic anemia were investigated, but no alternative cause was found. History excluded passive anti-D. D+, LW- cells were reactive, excluding anti-LW. Genotyping showed no evidence of a partial D genotype. Chart review revealed that the liver donor had a history of anti-D. A diagnosis of passenger lymphocyte syndrome was reached.
Although antibody-mediated hemolytic anemia has been reported to cause reticulocytopenia in the presence of marrow erythroid hyperplasia, this report of passenger lymphocyte syndrome causing a similar post-transplant anemia in association with reticulocytopenia is noteworthy.
当供体淋巴细胞与实体器官一同移植，并产生与受者红细胞（RBC）上抗原发生反应的同种抗体时，就会发生过客淋巴细胞综合征。通常，过客淋巴细胞综合征表现为免疫球蛋白G抗体介导的血管外溶血性贫血伴网织红细胞增多。供体常在移植前已被同种免疫。
一名34岁的O型、D抗原阳性男性，抗体筛查阴性，接受了来自O型、D抗原阴性供体的肝移植。术后第0天至第2天输注了20单位O型、D抗原阳性的红细胞。术后第7天，患者出现贫血、抗体筛查弱阳性以及洗脱液中抗D直接抗球蛋白试验阳性。术后第8天之后，启动了D抗原阴性输血方案。尽管有溶血的实验室证据，但最初两张外周血涂片显示裂红细胞或球形红细胞均未增多，网织红细胞计数降低，骨髓活检显示红系增生。最终，在一段时间的不依从药物治疗后贫血得以缓解；然而，直接抗球蛋白试验阳性一直持续到最后一次随访日期（术后第233天）。
对再生障碍性贫血的其他潜在病因进行了调查，但未发现其他病因。病史排除了被动抗D。D抗原阳性、LW抗原阴性的细胞呈反应性，排除了抗LW。基因分型未显示部分D基因型的证据。病历审查显示肝供体有抗D病史。得出过客淋巴细胞综合征的诊断。
虽然有报道称抗体介导的溶血性贫血在存在骨髓红系增生时可导致网织红细胞减少，但本报告中过客淋巴细胞综合征导致类似的移植后贫血并伴有网织红细胞减少值得关注。
