Ando Jun, Masuda Azuchi, Iizuka Kazuhide, Ochiai Tomonori, Takaku Tomoiku, Osawa Toshiya, Shimada Eiko, Watanabe Yoshihisa, Komatsu Norio, Osaka Akimichi
Department of Hematology, Juntendo University School of Medicine.
Rinsho Ketsueki. 2016;57(12):2507-2511. doi: 10.11406/rinketsu.57.2507.
A 77-year-old man with myelodysplastic syndrome suffered from duodenal perforation after undergoing endoscopic submucosal dissection (ESD) for treatment of duodenal cancer. He presented with hemorrhagic shock, peritonitis and disseminated intravascular coagulation (DIC), and received transfusions of red blood cells (RBC), fresh frozen plasma (FFP), γ-globulin and albumin (Alb). One month after the last RBC transfusion, prolonged thrombocytopenia was observed, and platelet concentrate (PC) was transfused. However, immediately after starting PC transfusion, he developed dyspnea, hypotension and rash, and was thus diagnosed as being in anaphylactic shock. Analysis of the patient's serum revealed absence of haptoglobin (Hp) and the presence of anti-Hp antibody. Further studies, using PCR detected Hp, yielded a diagnosis of congenital Hp deficiency. Thus, the anaphylactic shock was considered to have been induced by Hp in the transfused PC reacting with pre-existing anti-Hp antibodies. Thereafter, transfusions were safely carried out with the use of washed PC. Congenital Hp deficiency is relatively prevalent, and in such cases transfusions should be carried out using washed RBC, washed PC and congenital Hp deficiency donor derived FFP to avoid anaphylactic transfusion reactions. Transfusions would be even safer if production of congenital Hp deficiency donor derived PC were to be made available in the future.
一名77岁的骨髓增生异常综合征男性患者，因十二指肠癌接受内镜黏膜下剥离术（ESD）后发生十二指肠穿孔。他出现了失血性休克、腹膜炎和弥散性血管内凝血（DIC），并接受了红细胞（RBC）、新鲜冰冻血浆（FFP）、γ-球蛋白和白蛋白（Alb）的输血治疗。在最后一次输注RBC一个月后，观察到血小板减少持续存在，于是输注了血小板浓缩液（PC）。然而，在开始输注PC后不久，他出现了呼吸困难、低血压和皮疹，因此被诊断为过敏性休克。对患者血清的分析显示，血清中缺乏触珠蛋白（Hp）且存在抗Hp抗体。进一步使用聚合酶链反应（PCR）检测Hp的研究，确诊为先天性Hp缺乏症。因此，过敏性休克被认为是由于输注的PC中的Hp与预先存在的抗Hp抗体发生反应所致。此后，使用洗涤后的PC进行输血治疗，过程顺利。先天性Hp缺乏症相对较为常见，在这种情况下，应使用洗涤后的RBC、洗涤后的PC以及来自先天性Hp缺乏症供体的FFP进行输血，以避免过敏性输血反应。如果未来能够提供来自先天性Hp缺乏症供体的PC产品，输血将更加安全。
