Primary Central Nervous System Post-Transplantation Lymphoproliferative Disorder An International Primary Central Nervous System Lymphoma Collaborative Group Report
Primary Central Nervous System Post-Transplantation Lymphoproliferative Disorder An International Primary Central Nervous System Lymphoma Collaborative Group Report
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DOI：
10.1002/cncr.24834
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发表时间：
2010-02-15
期刊：
影响因子：
6.2
通讯作者：
Schiff, David
中科院分区：
文献类型：
--
作者：
Cavaliere, Robert;Petroni, Gina;Schiff, David
原发性中枢神经系统（CNS）移植后淋巴细胞增生性疾病（PCNSPTLD）是一种罕见的实体器官移植并发症。本研究的目的是确定该疾病的临床、放射学和病理特征，并探讨治疗对患者预后的影响。方法：作者回顾了国际原发性中枢神经系统淋巴瘤协作组参与机构的pns - ptld病例数据库。回顾性研究了34例经病理证实无系统性PTLD证据的PCNSPTLD患者。结果：从移植到诊断pns - ptld的中位时间为4.4年。疾病通常是多灶性的，涉及大脑的任何部位，但最常见于大脑半球，通常在皮层下白质或基底神经节。影像学上，所有病变均呈均匀增强或环状增强。大多数患者需要进行脑活检以确定诊断。大多数患者为单纯性eb病毒（EBV）阳性的b细胞源性疾病。无论何种治疗方式，有效率都很高，中位生存期为47个月。年龄是预测存活的唯一因素。结论：目前的研究表明，PCNS-PTLD是一种典型的ebv诱导的b细胞淋巴瘤，在许多患者中对治疗有反应，生存率较高。应强烈考虑采用积极的方法对组织进行诊断和化疗或放疗。癌症2010;116:863 - 70。(C) 2010年美国癌症协会
Primary central nervous system (CNS) post-transplantation lymphoproliferative disorder (PCNSPTLD) is a rare complication of solid organ transplantation. The objectives of this study were to define the clinical, radiologic, and pathologic features of this disease and to explore the impact of treatment on patient outcomes. METHODS: The authors reviewed the databases of participating institutions of the International Primary CNS Lymphoma Collaborative Group for cases of PCNS-PTLD. Thirty-four patients who had pathologically confirmed PCNSPTLD without evidence of systemic PTLD were investigated retrospectively. RESULTS: The median time from transplantation to diagnosis of PCNS-PTLD was 4.4 years. Disease usually was multifocal and involved any location of the brain but was most common in the cerebral hemispheres, usually in the subcortical white matter or basal ganglia. Radiographically, all lesions enhanced either homogenously or in a ring-enhancing pattern. Cerebral biopsy was required to establish diagnosis in most patients. Most patients had monomorphic, Epstein-Barr virus (EBV)-positive disease of B-cell origin. Response rates were high regardless of treatment type, and the median survival was 47 months. Age was the only factor predictive of survival. CONCLUSIONS: The current study demonstrated that PCNS-PTLD is typically an EBV-induced B-cell lymphoma that is responsive to treatment with favorable survival in many patients. An aggressive approach to tissue confirmation of diagnosis and treatment with chemotherapy or radiotherapy should be strongly considered. Cancer 2010;116:863-70. (C) 2010 American Cancer Society