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首页> 外文期刊>Neurosurgery >A Case Series of Primary Central Nervous System Posttransplantation Lymphoproliferative Disorder: Imaging and Clinical Characteristics
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A Case Series of Primary Central Nervous System Posttransplantation Lymphoproliferative Disorder: Imaging and Clinical Characteristics

机译：原发性中枢神经系统移植后淋巴细胞增生性疾病的病例系列：影像学和临床特征

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BACKGROUND: Primary central nervous system posttransplantation lymphoproliferative disorder (PCNS-PTLD) is a rare complication after solid organ transplantation (SOT). With increasing rates of SOT, PCNS-PTLD incidence is increasing. OBJECTIVE: To describe the characteristics of PCNS-PTLD patients requiring neuro-surgical intervention.METHODS: From 2000 to 2011, 10 patients with prior SOT underwent biopsy for evaluation of brain lesions and were diagnosed with PCNS-PTLD. Data collected included imaging characteristics, pathology, treatments administered, and survival outcomes.RESULTS: All patients had kidney transplantation, and 3 had concurrent pancreas transplantation. Median age at diagnosis was 49 years, with a median of 4.5 years from SOT to diagnosis (range, 1.8-11.4 years). Presenting symptoms most often included focal neurological deficits (n = 6), although several patients had nonspecific symptoms of headache and altered mental status. Brain lesions were generally multiple (n = 7), supratentorial (n = 8), and lobar or periventricular in distribution with ring enhancement. Diagnosis was established by stereotactic (n = 4) and open surgical (n = 6) biopsy. Treatments most frequently administered included reduction of immunosuppression (n = 10), dexamethasone (n = 10), rituximab (n .= 8), high-dose methotrexate (n = 3), and whole-brain radiotherapy (n = 6). Six patients remain alive without PCNS-PTLD relapse, including 4 patients who have sustained remissions beyond 2 years from diagnosis of PCNS-PTLD. Of 4 observed deaths, 1 was related to progressive PCNS-PTLD. CONCLUSION: PCNS-PTLD must be considered in the differential diagnosis of any patient with prior SOT presenting with an intracranial lesion. Histblogical diagnosis with brain biopsy is imperative, given the risk for opportunistic infections that may have similar imaging findings and presentation. Prognosis is variable, although long-term survival has-been reported.
机译：背景：原发性中枢神经系统移植后淋巴组织增生性疾病（PCNS-PTLD）是实体器官移植（SOT）后罕见的并发症。随着SOT率的增加，PCNS-PTLD的发病率也在增加。目的：描述需要神经外科干预的PCNS-PTLD患者的特点。方法：从2000年至2011年，对10例先有SOT的患者进行了活组织检查以评估脑部病变并被诊断为PCNS-PTLD。结果：所有患者均接受了肾脏移植，其中3例同时进行了胰腺移植。诊断时的中位年龄为49岁，从SOT到诊断的中位年龄为4.5岁（范围1.8-11.4岁）。表现出的症状通常包括局灶性神经功能缺损（n = 6），尽管一些患者有头痛的非特异性症状和精神状态改变。脑部病变通常为多发性（n = 7），幕上性（n = 8），且大叶或脑室分布环增强。通过立体定向（n = 4）和开放式手术（n = 6）活检来确定诊断。最常用的治疗包括降低免疫抑制（n = 10），地塞米松（n = 10），利妥昔单抗（n = 8），大剂量甲氨蝶呤（n = 3）和全脑放疗（n = 6）。 6例患者存活，没有PCNS-PTLD复发，其中4例从诊断PCNS-PTLD开始持续缓解超过2年。在观察到的4例死亡中，有1例与进行性PCNS-PTLD相关。结论：对于任何先有SOT颅内病变的患者，在鉴别诊断时必须考虑PCNS-PTLD。考虑到机会性感染的风险可能具有相似的影像学表现和表现，必须通过脑活检进行组织学诊断。尽管已有长期生存的报道，但预后是可变的。

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