血管内大B细胞淋巴瘤4例临床报告
Intravascular Large B-cell Lymphoma：a Report of 4 Cases

张景怡.血管内大B细胞淋巴瘤4例临床报告[J].内科急危重症杂志,2021,27(4):

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DOI：10.11768/nkjwzzzz20210407

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基金项目:国家自然科学基金青年基金（No：81500082）；华中科技大学2017年教学研究项目（No:108)；2020年第二临床学院教学研究基金立项项目（No:202035)

作者	单位	E-mail
张景怡	华中科技大学同济医学院附属同济医院	huanglifang627@163.com

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中文摘要:

目的：探讨血管内大B细胞淋巴瘤（IVLBCL）患者的临床特点及治疗方法。方法：回顾性分析华中科技大学同济医学院附属同济医院收治确诊的4例IVLBCL患者的临床特征、影像学资料、组织活检及免疫组织化学染色、并发症及治疗和预后。结果：4例病例典型临床表现包括不明原因的持续高热，常规抗生素、抗病毒、抗真菌治疗无效，有不同程度的贫血、肝功能不良、低蛋白血症，血清铁蛋白、乳酸脱氢酶（LDH）显著升高。4例免疫表型均表达CD20、CD79a、CD5、Bcl-2、PAX-5、MUM-1、ki67，血管内CD34阳性。病例2、4并发嗜血细胞综合征，称为血管内淋巴瘤病亚洲变异型(AIVL)。IVLBCL目前首选化疗方案为R-CHOP，除病例3外均接受了此方案化疗。病例4随后进行了自体造血干细胞移植+Car-T治疗，目前已完全缓解生存14个月，余3例分别在确诊后18、15、2个月死亡。结论：对于不明原因持续发热的患者，应详细体检，尽早行正电子发射型计算机断层显像（PET-CT）检查，对代谢异常增高的组织行病理学检查，并进行免疫组化染色及流式免疫分型，以便早期诊断或排除IVLBCL。自体造血干细胞移植+Car-T治疗有一定疗效。

英文摘要:

Objective: To investigate the clinical characteristics and treatment of intravascular large B-cell lymphoma(IVLBCL). Methods: The clinical features, imaging data, biopsy and immunohistochemical staining, complications, treatment and prognosis of 4 patients with IVLBCL in Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology were reviewed. Results: The typical clinical manifestations of the 4 cases included unexplained persistent high fever, ineffective conventional antibiotic, antiviral and antifungal treatment, anemia, liver dysfunction, hypoproteinemia, significant increase of serum ferritin and LDH. The immunophenotypes of 4 cases were positive for CD20, CD79a, CD5, Bcl-2, Pax-5, mum-1, Ki67, and intravascular CD34. Cases 2 and 4 were complicated with hemophagocytic syndrome, which was called Asian intravascular lymphoma (AIVL). At present, R-CHOP is the first choice for IVLBCL. All patients except case 3 received this chemotherapy. Case 4 was treated with autologous hematopoietic stem cell transplantation and Car-T. At present, the patient is still in complete remission. The other 3 patients died 18, 15 and 2 months after diagnosis. Conclusion: For patients with fever of unknown origin, detailed physical examination is very necessary, and PET-CT examination should be performed as early as possible. Biopsy should be performed for tissues with abnormal metabolism, and immunohistochemical staining and flow immunotyping should be performed for early diagnosis or exclusion of IVLBCL. Autologous hematopoietic stem cell transplantation and Car-T treatment has a certain effect.