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一例原发性双侧肾上腺淋巴瘤(PAL)伴中枢神经系统(CNS)受累。

A case of primary bilateral adrenal lymphoma (PAL) with central nervous system (CNS) involvement.

作者信息

Hahn Jee Sook, Choi Han Seok, Suh Chang Ok, Lee Woo Jung

机构信息

Department of Internal Medicine, Yonsei University College of Medicine, C.P.O. Box 8044, Seoul 120-752, Korea.

出版信息

Yonsei Med J. 2002 Jun;43(3):385-90. doi: 10.3349/ymj.2002.43.3.385.

Abstract

Primary adrenal lymphoma (PAL) is a very rare disease and usually does not have disease elsewhere, and if present, it is frequently extranodal. Several cases of PALs, with central nervous system (CNS) involvement, gastrointestinal tract or other endocrine organs involvement, have been reported in Western literature. We experienced a case of PAL with CNS involvement, which was previously unreported in Korea. The patient, a 61 year-old male, was admitted with left abdominal pain. After imaging study and needle aspiration biopsy (NAB), a bilateral primary adrenal lymphoma (large B-cell type), with bilateral hemorrhage, was confirmed. Combination chemotherapy, with CEOP regimen, was used. A follow-up abdomino- pelvic CT scan, after the second CEOP chemotherapy, showed the previous adrenal masses had nearly disappeared. However, about 2 months after diagnosis (day 52), he showed recent memory loss, obtundation, disorientation and drowsy mental status. The brain MRI revealed multifocal scattered lesions with increased signal intensity. The result of a brain biopsy was diffuse large B-cell lymphoma. After 5(1/2) cycles of chemotherapy, with CEOP regimen, and whole brain radiotherapy, he is still surviving 6 months later, and has become alert with nearly normalized cognitive function.

摘要

原发性肾上腺淋巴瘤(PAL)是一种非常罕见的疾病,通常在其他部位无病变,若有其他部位病变,则常为结外病变。西方文献中已报道了几例伴有中枢神经系统(CNS)受累、胃肠道或其他内分泌器官受累的PAL病例。我们遇到了一例伴有CNS受累的PAL病例,这在韩国此前尚未见报道。该患者为一名61岁男性,因左腹痛入院。经影像学检查和针吸活检(NAB),确诊为双侧原发性肾上腺淋巴瘤(大B细胞型),伴有双侧出血。采用CEOP方案进行联合化疗。第二次CEOP化疗后进行的腹部盆腔CT随访扫描显示,先前的肾上腺肿块几乎消失。然而,在诊断后约2个月(第52天),他出现了近期记忆力减退、意识模糊、定向障碍和嗜睡的精神状态。脑部MRI显示多灶性散在病变,信号强度增加。脑活检结果为弥漫性大B细胞淋巴瘤。在采用CEOP方案进行5(1/2)周期化疗和全脑放疗后,6个月后他仍然存活,并且已经恢复警觉,认知功能几乎恢复正常。

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