Department of Medicine, The Royal Melbourne Hospital, Melbourne, VIC, Australia
Department of Medicine, Albury Wodonga Health, Albury, NSW, Australia.
BMJ Case Rep. 2021 Nov 2;14(11):e244069. doi: 10.1136/bcr-2021-244069.
Intravascular large B-cell lymphoma (ILBCL) is a subtype of non-Hodgkin's large B-cell lymphoma that is characterised by neoplastic lymphocyte proliferation within the lumen of small blood vessels, which may occur without an extracellular tumour mass or peripheral blood involvement. This report highlights some of the diagnostic issues for ILBCL, and how it can be approached. The two cases described below highlight two significantly different presentations, one with predominately neurological phenomena, and the other with fever of unknown origin for investigation. Both patients were managed with chemotherapy and intercalated intrathecal chemotherapy, with good clinical outcomes, without further evidence of clinical relapse. These cases along with a review of the literature highlight the key learning points in the difficulties in the diagnosis of this condition, and the appropriate use of random skin biopsy in patient suspected of having ILBCL, such as those with constitutional symptoms with otherwise negative malignancy screening, and unexplained neurological phenomena, especially if recurrent in nature.
血管内大 B 细胞淋巴瘤(ILBCL)是非霍奇金大 B 细胞淋巴瘤的一种亚型,其特征为小血管腔内部瘤性淋巴细胞增殖,可能没有细胞外肿瘤块或外周血受累。本报告强调了 ILBCL 的一些诊断问题,以及如何处理它。下面描述的两个病例突出了两种截然不同的表现,一种主要表现为神经现象,另一种表现为不明原因发热进行调查。两名患者均接受了化疗和鞘内化疗联合治疗,临床结局良好,没有进一步的临床复发证据。这些病例以及对文献的回顾强调了在诊断这种疾病时存在的困难的关键学习点,以及在疑似患有 ILBCL 的患者中适当使用随机皮肤活检的重要性,例如那些有全身症状且恶性肿瘤筛查阴性的患者,以及不明原因的神经现象,尤其是反复发作的患者。