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一名免疫缺陷患者中枢神经系统复发伯基特淋巴瘤的临床病理及神经放射学表现:1例极为罕见的病例,经GMALL-B-ALL/NHL2002和R-MIV方案治疗后两次实现完全缓解

Clinicopathological and neuroradiological presentation in an immuno-deficiency patient with central nervous system recurrence of Burkitt lymphoma: an extremely rare case with complete remission achieved twice after GMALL-B-ALL/NHL2002 and R-MIV protocols.

作者信息

Glinka Piotr, Sobstyl Michał, Rymkiewicz Grzegorz, Karamon Karol, Skowronek-Płachta Monika, Acewicz Albert, Błachnio Katarzyna, Konecki Robert, Chmielewski Marcin

机构信息

Department of Neurosurgery, Institute of Psychiatry and Neurology, Warsaw, Poland.

Flow Cytometry Laboratory, Department of Cancer Pathomorphology, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland.

出版信息

Folia Neuropathol. 2025;63(2):198-208. doi: 10.5114/fn.2025.152574.

Abstract

Burkitt lymphoma (BL) is a specific, rare (1-2% of all cases of lymphoma) and aggressive type of B-cell non-Hodgkin lymphoma. If BL recurs during treatment, it usually becomes drug-resistant to subsequent treatment regimens and the prognosis is very poor. We present an extremely rare case of recurrent BL infiltrating the central nervous system (CNS) in a 27-year-old HIV-infected patient who had completed GMALL-B-ALL/NHL2002 protocol treatment 4 months earlier. Radiological and neuropathological examinations of cerebral recurrence of BL, followed by specialized oncological treatment, were discussed. Currently, with antiretroviral therapy and intensive immunochemotherapy for the immunodeficiency-associated BL variant, disease recurrence in the CNS is extremely rare. The magnetic resonance imaging (MRI) findings and laboratory tests are unspecific, as shown in the present case. The final diagnosis can be established properly by performing image-guided stereotactic biopsy or flow cytometry with cytology examination of cerebrospinal fluid. After confirmation of isolated cerebral recurrence of BL, the patient received intensive treatment according to the R-MIV protocol and achieved a second complete remission.

摘要

伯基特淋巴瘤(BL)是一种特殊的、罕见的(占所有淋巴瘤病例的1%-2%)侵袭性B细胞非霍奇金淋巴瘤。如果BL在治疗期间复发,通常会对后续治疗方案产生耐药性,预后很差。我们报告了一例极其罕见的复发性BL病例,该病例发生在一名27岁的HIV感染患者身上,其4个月前已完成GMALL-B-ALL/NHL2002方案治疗,复发性BL浸润中枢神经系统(CNS)。本文讨论了BL脑复发的影像学和神经病理学检查,以及随后的专科肿瘤治疗。目前,对于免疫缺陷相关的BL变异型,采用抗逆转录病毒疗法和强化免疫化疗后,CNS疾病复发极为罕见。如本病例所示,磁共振成像(MRI)结果和实验室检查并无特异性。通过图像引导立体定向活检或脑脊液细胞学检查的流式细胞术可正确做出最终诊断。在确诊为孤立性BL脑复发后,患者根据R-MIV方案接受了强化治疗,并实现了第二次完全缓解。

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