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原发性中枢神经系统 EBV 阳性移植后淋巴组织增生性疾病伴多形性和经典霍奇金淋巴瘤特征:一例病例报告及文献复习。

Primary CNS EBV-positive post-transplant lymphoproliferative disorder with polymorphic and classic Hodgkin lymphoma features: A case report and literature review.

出版信息

Clin Neuropathol. 2023 Jul-Aug;42(4):161-167. doi: 10.5414/NP301526.

Abstract

Post-transplant lymphoproliferative disorders (PTLD) are typically Epstein-Barr virus (EBV)-associated lymphoid or plasmacytic proliferations that occur when immunosuppressed after transplantation. Only 2 cases of primary central nervous system (PCNS) classic Hodgkin lymphoma PTLD and 1 case of PCNS Hodgkin lymphoma-like PTLD have been previously reported. A 59-year-old male presented with malaise, headaches, and dizziness; neuroimaging revealed a 1.7-cm right cerebellar mass and a 0.6-cm right frontal mass. Microscopic examination demonstrated a perivascular and parenchymal polymorphous infiltrate composed of lymphocytes (CD3-positive T cells and CD20-positive B cells), plasma cells, and macrophages. Focally, macrophages had a spindled morphology with a fascicular arrangement amounting to poorly formed granulomata. Mitoses were seen. Scattered large atypical cells were visualized with irregular hyperchromatic nuclei, reminiscent of lacunar cells, mononuclear Hodgkin and binucleate Reed-Sternberg (RS) cells. EBV in situ highlighted a significant number of small lymphoid cells as well as many large atypical forms. Large atypical cells were seen to co-express CD15 and CD30. To our knowledge, this is the first such case with hybrid polymorphic PTLD and classic Hodgkin lymphoma features and the first such case to arise following liver transplantation. This case highlights the histological and immunophenotypic spectrum of these lymphoid proliferations and the resulting challenges in diagnosis and definitive subtyping.

摘要

移植后淋巴组织增生性疾病(PTLD)通常是 EBV 相关的淋巴或浆细胞增生,发生在移植后免疫抑制时。先前仅报道过 2 例原发性中枢神经系统(PCNS)经典霍奇金淋巴瘤 PTLD 和 1 例 PCNS 霍奇金淋巴瘤样 PTLD。一名 59 岁男性出现不适、头痛和头晕;神经影像学显示 1.7cm 右侧小脑肿块和 0.6cm 右侧额叶肿块。显微镜检查显示血管周围和实质多形性浸润,由淋巴细胞(CD3 阳性 T 细胞和 CD20 阳性 B 细胞)、浆细胞和巨噬细胞组成。局部巨噬细胞呈梭形形态,呈束状排列,形成不成形的肉芽肿。有丝分裂可见。散在的大异型细胞具有不规则的嗜碱性核,类似于腔隙细胞、单核霍奇金和双核 Reed-Sternberg(RS)细胞。EBV 原位显示大量小淋巴细胞和许多大异型形式。大异型细胞被观察到共同表达 CD15 和 CD30。据我们所知,这是首例具有混合多形性 PTLD 和经典霍奇金淋巴瘤特征的病例,也是首例发生在肝移植后的病例。该病例突出了这些淋巴增生的组织学和免疫表型谱,以及在诊断和明确亚型方面的挑战。

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