Chroboczek T, Lazaro E, Greib C, Parrens M, Dilhuydi M S, Pellegrin J L, Viallard J F
Service de médecine interne et maladies infectieuses, hôpital Haut-Lévèque, CHU de Bordeaux, avenue de Magellan, 33604 Pessac cedex, France.
Rev Med Interne. 2012 May;33(5):250-8. doi: 10.1016/j.revmed.2011.12.010. Epub 2012 Feb 4.
Intravascular large B cell lymphoma (IVLBCL) is a rare and aggressive variant of non-Hodgkin's lymphoma, characterized by multifocal proliferation of lymphoma cells exclusively within small and medium blood vessels. IVLBCL can be systemic and quite polymorphic, which often makes it difficult to diagnose, thus delaying appropriate treatment.
We report three patients of atypical IVLBCL, through the study of which we overview recent knowledge about IVLBCL.
The first patient initially presented with peripheral thrombocytopenia and splenic destruction of platelets, later completed with an interstitial pulmonary syndrome. The second patient, of African origin, we believe is the first case of a black patient with IVLBCL described in the medical literature. The third belongs to the rare group of occidental patients that present an IVLBCL associated with a hemophagocytic syndrome.
Intravascular large B cell lymphoma is a systemic and polymorphic disease. Awareness of this entitiy should allow rapid and appropriate management.
血管内大B细胞淋巴瘤（IVLBCL）是非霍奇金淋巴瘤的一种罕见且侵袭性变体，其特征为淋巴瘤细胞仅在中小血管内多灶性增殖。IVLBCL可为全身性且表现多样，这常使其难以诊断，从而延误适当治疗。
我们报告3例非典型IVLBCL患者，通过对其研究概述了关于IVLBCL的最新知识。
首例患者最初表现为外周血小板减少及脾脏血小板破坏，随后出现间质性肺综合征。第二例患者来自非洲，我们认为是医学文献中描述的首例患有IVLBCL的黑人患者。第三例属于罕见的西方患者群体，其患有与噬血细胞综合征相关的IVLBCL。
血管内大B细胞淋巴瘤是一种全身性且表现多样的疾病。认识到这一实体应有助于快速且适当地进行管理。
