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惰性 B 细胞淋巴瘤伴大量胸腔积液为首发表现 1 例罕见病例。

A rare case of indolent B cell lymphoma with massive pleural effusion as the initial presentation.

机构信息

Department of Geriatrics, Peking University First Hospital, Beijing, China.

Department of Hematology, Peking University First Hospital, Beijing, China.

出版信息

Ann Palliat Med. 2021 Jun;10(6):7033-7041. doi: 10.21037/apm-20-1480. Epub 2020 Nov 18.

DOI:10.21037/apm-20-1480
PMID:33222449
Abstract

Two major type of lymphoma involve the pleura as primary neoplasms: primary effusion lymphoma (PEL) in the setting of human immunodeficiency virus (HIV) infection, and pyothorax-associated lymphomas (PAL), with a strong Epstein-Barr virus (EBV) or pyothorax association. However, indolent B cell lymphoma initially manifested as pleural effusion is an extremely rare clinical occurrence. In this study, we report a case of 52-year-old woman with no history of HIV infection or pyothorax, who only manifested long-term massive bilateral pleural effusion. This patient was characterized by lymphocytic pleural effusion, which had been misdiagnosed as tuberculous hydrothorax. A total of 75 liters of pleural effusion were drained over a two-year period. After admission to our hospital, we performed flow cytometry of pleural effusion and revealed proliferation of B lymphocytes with abnormal immune markers consistent with marginal zone B cell phenotype, although lymphocyte morphology is normal. Flow cytometry on bone marrow revealed ckappa restrictive expression on B cells further, which indicating small B cell lymphoma. The right-side pleural fluid was encapsulated after thoracoscopy and the patient's symptom were relieved. Considering the foreseeable side effects of chemotherapeutic drugs and indolent potential of the small B cell lymphoma, the patient opted not to undergo further treatment. Follow up was done 1, 3 and 6 months after discharge, the depths of bilateral pleural effusion on ultrasound stabilized around 3 cm without thoracentesis. This case is thought to be an unusual presentation because the pleural lymphoma occurred on an immunocompetent adult woman and the type was small B cell lymphoma, which were totally different from PEL or PAL. We also describe the use of flow cytometry to effectively diagnose the unexplained pleural effusion, and discuss the findings using relevant previously reported literature. Overall, our findings provide new insights to deal with unexplained pleural effusion and the value of flow cytometry in diagnosis.

摘要

两种主要类型的淋巴瘤涉及胸膜作为原发性肿瘤

人类免疫缺陷病毒 (HIV) 感染背景下的原发性渗出性淋巴瘤 (PEL),以及与胸脓关联的淋巴瘤 (PAL),与强烈的 Epstein-Barr 病毒 (EBV) 或胸脓关联。然而,最初表现为胸腔积液的惰性 B 细胞淋巴瘤是一种极其罕见的临床现象。在本研究中,我们报告了一例 52 岁女性病例,无 HIV 感染或胸脓病史,仅表现为长期大量双侧胸腔积液。该患者的特征是淋巴细胞性胸腔积液,曾误诊为结核性胸水。在两年期间共引流了 75 升胸腔积液。患者入住我院后,我们对胸腔积液进行了流式细胞术检测,结果显示 B 淋巴细胞增殖,具有异常免疫标志物,符合边缘区 B 细胞表型,尽管淋巴细胞形态正常。骨髓流式细胞术进一步显示 B 细胞 ckappa 受限表达,提示小 B 细胞淋巴瘤。胸腔镜检查后右侧胸腔积液被包裹,患者症状缓解。考虑到化疗药物的可预见副作用和小 B 细胞淋巴瘤的惰性潜能,患者选择不进行进一步治疗。出院后 1、3 和 6 个月进行随访,超声显示双侧胸腔积液深度稳定在 3 厘米左右,无需胸腔穿刺。由于胸膜淋巴瘤发生在免疫功能正常的成年女性身上,且类型为小 B 细胞淋巴瘤,与 PEL 或 PAL 完全不同,因此认为该病例是一种不常见的表现。我们还描述了使用流式细胞术有效诊断不明原因胸腔积液,并使用相关已发表文献讨论了研究结果。总的来说,我们的研究结果为处理不明原因胸腔积液提供了新的见解,并展示了流式细胞术在诊断中的价值。

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