Department of Neurosciences and Behavioral Sciences, Ribeirão Preto Medical School, University of São Paulo, São Paulo, Brazil.
Department of Medical Imaging, Hematology, and Clinical Oncology, Ribeirão Preto Medical School, University of São Paulo, São Paulo, Brazil.
J Neuroimmunol. 2024 Nov 15;396:578459. doi: 10.1016/j.jneuroim.2024.578459. Epub 2024 Sep 11.
Intravascular large B-cell lymphoma (IVLBCL) is a rare hematological malignancy where its development in the intravascular environment is the main characteristic. Despite its ability to affect multiple organic systems, there is a tropism for the central nervous system, which may be related to several clinical syndromes, making this condition a great mimic and consequently a diagnostic challenge. Rapidly progressive dementia may be one of the presenting phenotypes of IVLBCL. This case report aims to highlight the main red flags, such as sustained elevation of lactate dehydrogenase, organomegaly and specific lesions with vasculitis-like bleeding, all that can be used as clinical clues to direct the differential diagnosis. In addition, it reinforces the role of early brain biopsy in this context, since IVLBCL is a treatable disease.
血管内大 B 细胞淋巴瘤（IVLBCL）是一种罕见的血液系统恶性肿瘤，其在血管内环境中的发展是主要特征。尽管它能够影响多个器官系统，但它对中枢神经系统有一定的倾向性，这可能与几种临床综合征有关，使这种情况成为一个很好的模拟，因此诊断具有挑战性。进行性快速痴呆可能是 IVLBCL 的一种表现表型。本病例报告旨在强调主要的危险信号，如乳酸脱氢酶持续升高、器官肿大和具有血管炎样出血的特定病变，这些都可以作为临床线索来指导鉴别诊断。此外，它还强调了在这种情况下早期脑活检的作用，因为 IVLBCL 是一种可治疗的疾病。
