Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, Japan.
Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, Japan.
Int J Infect Dis. 2019 Nov;88:31-33. doi: 10.1016/j.ijid.2019.07.039. Epub 2019 Aug 6.
A 69-year-old man who underwent cord blood transplantation seven years ago was admitted because of fever, elevated liver enzymes and thrombocytopenia. Bone marrow aspirate revealed hemophagocytic lymphohistiocytosis. Viral capsid antigen (VCA)-immunoglobulin (Ig) G, VCA-IgM, VCA-IgA, Epstein-Barr virus nuclear antigen-IgG, early antigen-diffuse-type and restricted-type (EA-DR) IgG, and EA-DR IgA titers were 2560, <10, 10, 40, 40, and <10, respectively. Real-time polymerase chain reaction assay of peripheral whole blood for Epstein-Barr virus-deoxyribonucleic acid (EBV-DNA) revealed 240,000 copies/μg DNA. Flow cytometric in situ hybridization assay confirmed that EBV-infected cells were NK-cells. Clonality evaluation by Southern blot assay of EBV-DNA terminal repeats proved to be bi-clonal. Accordingly, we made a diagnosis of NK-cell post-transplant lymphoproliferative disease with chronic active EBV infection-like clinical findings (CAEBV-like NK-cell PTLD). Although CAEBV-like PTLD is extremely rare, its prognosis seems to be very poor. The disease should be considered in such patients who present persistent or recurrent infectious mononucleosis-like symptoms after transplantation.
一位 69 岁男性，七年前接受过脐带血移植，因发热、肝酶升高和血小板减少而入院。骨髓抽吸物显示噬血细胞性淋巴组织细胞增生症。病毒衣壳抗原（VCA）-免疫球蛋白（Ig）G、VCA-IgM、VCA-IgA、EB 病毒核抗原-IgG、早期抗原-弥散型和限制型（EA-DR）IgG 和 EA-DR IgA 的滴度分别为 2560、<10、10、40、40 和 <10。外周全血 EBV-DNA 的实时聚合酶链反应检测显示 EBV-DNA 为 240,000 拷贝/μg DNA。EBV 感染细胞的流式细胞术原位杂交检测证实为 NK 细胞。通过 EBV-DNA 末端重复序列的 Southern 印迹分析进行的克隆性评估证明是双克隆的。因此，我们诊断为 NK 细胞移植后淋巴组织细胞增生性疾病伴慢性活动性 EBV 感染样临床表现（CAEBV 样 NK 细胞 PTLD）。尽管 CAEBV 样 PTLD 极为罕见，但预后似乎非常差。对于移植后出现持续性或复发性传染性单核细胞增多症样症状的患者，应考虑该病。
