Adult anaplastic large cell lymphoma involving the central nervous system: a rare clinical scenario
Adult anaplastic large cell lymphoma involving the central nervous system: a rare clinical scenario
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累及中枢神经系统的成人间变性大细胞淋巴瘤：罕见的临床病例
DOI：
10.1007/s00277-010-1074-2
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发表时间：
2011
影响因子：
3.5
通讯作者：
Ling Zhang
中科院分区：
文献类型：
--
作者：
Xiaohui Zhang;M. Bui;J. Caracciolo;T. Field;Ling Zhang
尊敬的编辑，间变性大细胞淋巴瘤 (ALCL) 是一种罕见的 T 细胞非霍奇金淋巴瘤，具有独特的病理特征，其特征是表达 CD30 的大多形性淋巴细胞，伴或不伴间变性淋巴瘤激酶 (ALK) 基因重排。该疾病通常扩散至骨髓、皮肤、肺、肝脏，很少扩散至其他部位[1]。 ALCL很少发生在中枢神经系统（CNS），其中大多数是儿童或免疫功能低下患者中报道的原发性ALCL[2]。继发性中枢神经系统受累的 ALCL 极为罕见[3]。在此，我们介绍了两名继发中枢神经系统受累的 ALCL 成年患者，均接受标准化疗和自体造血干细胞移植 (auto-SCT) 治疗，且两名患者的生存期相对较长。迄今为止，仅报告了另外三例类似病例，并且在没有进行自动 SCT 治疗的情况下，所有病例的生存期都要短得多。一名 27 岁男性出现纵隔肿块和全身淋巴结肿大，随后被诊断为 ALK 阳性 ALCL。肿瘤细胞表达CD30和ALK。他接受了环磷酰胺、羟基柔红霉素（多柔比星）、Oncovin（长春新碱）和泼尼松/泼尼松龙 (CHOP) 化疗以及预防性大剂量鞘内甲氨蝶呤三个周期。初次诊断 ALCL 一年后，患者出现癫痫发作。对比增强的脑部 MRI 显示顶叶和枕叶区域弥漫性水肿。除了弥漫性软脑膜强化和硬脑膜受累外，小脑幕上方和下方也有实质疾病的证据（图1a和b）。脑脊液 (CSF) 细胞离心涂片检查显示，在少量粒细胞和巨噬细胞的背景下存在大量间变性淋巴细胞。脑脊液流式细胞术发现表达 CD30 和 CD4 的异常 T 细胞，但其他泛 T 细胞标记物（CD3、CD5 和 CD7）丢失。对 CSF DNA 样本的 T 细胞基因重排分析检测到一个显着但暗淡的 T 细胞受体 γ 单克隆峰。此时外周血中未发现循环淋巴瘤细胞。患者接受鞘内甲氨蝶呤和阿糖胞苷治疗，然后进行全脑放射治疗。尽管并发了严重的脑病和脑梗塞，导致周边视力丧失，但病情得到了很好的控制。五年后，他出现严重腹痛和腹泻。 PET/CT 扫描显示十二指肠远端代谢亢进，活检结果与复发性 ALCL 一致
Dear Editor, Anaplastic large cell lymphoma (ALCL) is a rare T-cell non-Hodgkin lymphoma with distinct pathologic features characterized by CD30 expressing large pleomorphic lymphoid cells with or without anaplastic lymphoma kinase (ALK) gene rearrangement. The disease commonly spreads to bone marrow, skin, lung, liver, and, rarely, other sites [1]. ALCL rarely occurs in the central nervous system (CNS), of which most are primary ALCL reported in pediatric or immunocompromised patients [2]. ALCL with secondary CNS involvement is extremely rare [3]. Here, we present two adult patients having ALCL with secondary CNS involvement, both treated with standard chemotherapy and autologous hematopoietic stem cell transplant (auto-SCT), and both patients had a relatively long survival. To date, only three other similar cases have been reported and all had much shorter survival with no auto-SCT treatment. A 27-year-old man who presented with a mediastinal mass and general lymphoadenopathy was subsequently diagnosed with ALK-positive ALCL. The tumor cells expressed CD30 and ALK. He received cyclophosphamide, hydroxydaunorubicin (doxorubicin), Oncovin (vincristine), and prednisone/prednisolone (CHOP) chemotherapy along with prophylactic high-dose intrathecal methotrexate for three cycles. One year after the initial diagnosis of ALCL, the patient developed seizures. Contrast-enhanced brain MRI revealed diffuse edema in both the parietal and occipital regions. There was also evidence of parenchymal disease both above and below the tentorium in addition to diffuse pial enhancement and dural involvement (Fig. 1a and b). Cerebrospinal fluid (CSF) cytospin examination revealed a population of large anaplastic lymphoid cells in the background of few granulocytes and macrophages. Flow cytometry of the CSF identified abnormal T cells expressing CD30 and CD4 with loss of other pan-T-cell markers (CD3, CD5, and CD7). A T-cell gene rearrangement analysis of the CSF DNA sample detected a prominent but dim T-cell receptor gamma monoclonal peak. No circulating lymphoma cells were identified in the peripheral blood at that time point. The patient received intrathecal methotrexate and cytarabine followed by wholebrain radiation. His disease was well controlled although it was complicated with severe encephalopathy and cerebral infarct, which caused peripheral vision loss. Five years later, he developed severe abdominal pain and diarrhea. PET/CT scan revealed hypermetabolic activity in the distal duodenum, and biopsy was consistent with recurrent ALCL