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原发性中枢神经系统其他医源性免疫缺陷相关淋巴组织增生性疾病表现为骨外浆细胞瘤,具有进行性临床病程:病例报告及文献复习。

Primary central nervous system other iatrogenic immunodeficiency-associated lymphoproliferative disorders presenting as extraosseous plasmacytoma with a progressive clinical course: A case report and literature review.

机构信息

Department of Neurosurgery, Yokohama City Minato Red Cross Hospital, Yokohama, Japan.

Department of Pathology, Yokohama City Minato Red Cross Hospital, Yokohama, Japan.

出版信息

Neuropathology. 2023 Apr;43(2):151-157. doi: 10.1111/neup.12863. Epub 2022 Sep 5.

DOI:10.1111/neup.12863
PMID:36065518
Abstract

Other iatrogenic immunosuppressive-associated lymphoproliferative disorders (OIIA-LPDs) rarely occur in the central nervous system (CNS). Additionally, they almost always present as lymphoma and withdrawal by cessation of immunosuppressive treatment. We report a case of primary CNS OIIA-LPD that presented as extraosseous plasmacytoma (EP) with a progressive clinical course in spite of immunosuppressive treatment cessation. A 78-year-old man with a history of rheumatoid arthritis (RA) presented with a month-long headache. Magnetic resonance imaging showed mass lesions in the left temporal lobe, left middle fossa, and intradural cervical spine. The left temporal lesion was resected and diagnosed as EP histologically, and OIIA-LPD presented as plasmacytoma integrally due to his history of immunosuppressive treatment using tacrolimus for RA. Despite immunosuppressive treatment cessation, OIIA-LPD lesions did not regress but, on the contrary, showed a progressive clinical course. Considering his advanced age and renal dysfunction, postoperative treatment with radiation and moderate chemotherapy using prednisolone were administrated. Subsequently, the disease state stabilized, and the patient had a Karnofsky performance status score of 90 for 6 months; however, the tumor recurred with meningeal dissemination, and he died 8 months after treatment. Types of OIIA-LPD onset as EP and its progressive clinical course resistant to cessation of immunosuppressive treatment are rare. Moreover, this OIIA-LPD disease state worsened despite its radiosensitivity. We believe the progressive clinical course of this OIIA-LPD case with its high cell proliferation is similar to Epstein-Barr virus negative plasmablastic lymphoma, which could lead to a poor outcome.

摘要

其他医源性免疫抑制相关淋巴组织增生性疾病(OIIA-LPD)在中枢神经系统(CNS)中很少发生。此外,它们几乎总是表现为淋巴瘤,并在停止免疫抑制治疗后消退。我们报告了一例原发性中枢神经系统 OIIA-LPD 的病例,尽管停止了免疫抑制治疗,但它表现为进展性临床病程的骨外浆细胞瘤(EP)。一名 78 岁男性,有类风湿关节炎(RA)病史,出现长达一个月的头痛。磁共振成像显示左颞叶、左中颅窝和硬脑膜内颈椎的肿块病变。左颞叶病变被切除,组织学诊断为 EP,由于他使用他克莫司治疗 RA 的免疫抑制治疗史,OIIA-LPD 表现为浆细胞瘤。尽管停止了免疫抑制治疗,但 OIIA-LPD 病变并未消退,反而表现出进行性临床病程。考虑到他的年龄较大和肾功能不全,术后给予了放射治疗和中等强度化疗,使用泼尼松龙。随后,疾病状态稳定,患者的 Karnofsky 表现状态评分为 90,持续了 6 个月;然而,肿瘤复发并伴有脑膜播散,治疗 8 个月后死亡。OIIA-LPD 以 EP 为首发表现且对免疫抑制治疗停药具有进展性临床病程的类型较为罕见。此外,尽管该 OIIA-LPD 具有放射敏感性,但疾病状态仍恶化。我们认为这种 OIIA-LPD 病例的进行性临床病程和高细胞增殖类似于 EBV 阴性浆母细胞淋巴瘤,这可能导致不良预后。

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