Abstract:
Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus-associated systemic angiocentric and angiodestructive lymphoproliferative disorder. It commonly involves the lungs and can also affect the skin, liver, kidney, and central nervous system. It can rarely occur in the spine, however, the details are unclear. We performed a systematic review of published cases (including our 1 case) of spinal LYG. We performed a systematic search of studies in English on spinal LYG, focusing on its clinical features, imaging, and treatments, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines on the PubMed database. We identified 14 patients from the literature. We also found 1 case of isolated cervical LYG (grade 3) who was treated with steroid and radiation therapy for the spinal lesion after pathologic diagnosis. We performed a pooled analysis of these 15 cases. The mean age was 43.4 years, and 13 of the 15 patients were male. Brain lesions were present in 11 of 12 intramedullary spinal lesions, and only 1 was an isolated spinal LYG case. Regarding the diagnostic methods, 1 case was not described. Of the 14 cases described, 12 patients underwent biopsies (7 brain, 4 lung, and 1 spinal cord lesion) and 2 underwent surgical removal for an extramedullary lesion. In the overall prognosis from a mean follow-up period of 21.6 months, 4 patients died despite several treatments. Spinal LYG, particularly isolated spinal LYG, is rare. Thus further accumulation of cases may be necessary to better understand its characteristics.
摘要:
淋巴瘤样肉芽肿病(LYG)是一种罕见的与EB病毒相关的全身性血管中心和血管破坏性淋巴增生性疾病。它通常累及肺部,也会影响皮肤,肝脏,肾,和中枢神经系统。它很少发生在脊柱,然而,细节还不清楚。我们对已发表的脊髓LYG病例(包括我们的1例)进行了系统评价。我们对脊柱LYG的英语研究进行了系统的搜索,关注其临床特征,成像,和治疗,根据PubMed数据库上系统审查和荟萃分析指南的首选报告项目。我们从文献中确定了14名患者。我们还发现了1例孤立的宫颈LYG(3级),在病理诊断后对脊柱病变进行了类固醇和放射治疗。我们对这15例病例进行了汇总分析。平均年龄是43.4岁,15例患者中有13例为男性。12个脊髓髓内病变中有11个存在脑部病变,只有1例是孤立的脊髓LYG病例。关于诊断方法,1例没有描述。在描述的14个案例中,12例患者接受了活检(7例脑,4肺,1例脊髓病变)和2例髓外病变手术切除。在平均21.6个月的随访期的总体预后中,尽管经过多次治疗,仍有4名患者死亡。脊髓LYG,特别是孤立的脊髓LYG,是罕见的。因此,可能需要进一步积累案例,以更好地了解其特征。