Yokoyama Naosuke, Kanbayashi Takamichi, Kobayashi Shunsuke, Ishida Tsuyoshi, Sonoo Masahiro
Department of Neurology, Teikyo University School of Medicine.
Department of Pathology, Teikyo University School of Medicine.
Rinsho Shinkeigaku. 2021 Nov 24;61(11):750-755. doi: 10.5692/clinicalneurol.cn-001623. Epub 2021 Oct 16.
We report a 66-year-old man with primary central nervous system post-transplant lymphoproliferative disorder (PCNS-PTLD). He had received a living-donor kidney transplantation at the age of 64 years. Although he had a good postoperative course by continuing to take oral immunosuppressive agents, he was admitted to our hospital for subacute cognitive impairment and urinary retention two years after the transplantation. Brain MRI revealed high-intensity lesions on FLAIR and T-weighted images in the left parietal operculum, deep white matter around the anterior horn of the lateral ventricle, and the genu and splenium of the corpus callosum. A part of these lesions showed ring enhancement. The cerebrospinal fluid examination revealed lymphocytic pleocytosis, elevation of protein level, and mild hypoglycorrhachia. Blood tests showed no abnormalities except for positive serum VCA-IgG antibody of Epstein-Barr virus. A brain biopsy was performed and diagnosis of PCNS-PTLD was made. There was no evidence of systemic PTLD. We reduced the dose of immunosuppressive agents and started the initial treatment with methylprednisolone pulse therapy. The patient showed a partial response to the treatment and transferred to another hospital for subsequent chemotherapy. PTLD is an important post-transplant complication that can affect the patient's prognosis. The incidence of PTLD is increasing with the growing numbers of transplantations and older age of donors and recipients. Although CNS involvement is known to be rare, PCNS-PTLD is an important differential diagnosis when symptoms of CNS origin develop in post-transplant patients.
我们报告了一名66岁患有原发性中枢神经系统移植后淋巴组织增生性疾病(PCNS-PTLD)的男性。他在64岁时接受了活体供肾移植。尽管通过持续服用口服免疫抑制剂术后恢复良好,但移植两年后他因亚急性认知障碍和尿潴留入住我院。脑部MRI显示,在液体衰减反转恢复序列(FLAIR)和T加权图像上,左侧顶叶岛盖、侧脑室前角周围深部白质以及胼胝体膝部和压部有高强度病变。其中部分病变呈环形强化。脑脊液检查显示淋巴细胞增多、蛋白水平升高以及轻度脑脊液低糖。血液检查除了EB病毒血清VCA-IgG抗体呈阳性外无异常。进行了脑活检,诊断为PCNS-PTLD。没有系统性PTLD的证据。我们减少了免疫抑制剂的剂量,并开始用甲泼尼龙冲击疗法进行初始治疗。患者对治疗有部分反应,随后转至另一家医院进行后续化疗。PTLD是一种重要的移植后并发症,可影响患者预后。随着移植数量的增加以及供体和受体年龄的增长,PTLD的发病率正在上升。尽管已知中枢神经系统受累罕见,但当移植后患者出现中枢神经系统起源的症状时,PCNS-PTLD是重要的鉴别诊断。