Maisey N R, Waters J S, Collins D, Schofield J, Hill M E
Department of Medicine, Royal Marsden Hospital, Fulham Road, London SW3 6JJ, UK.
Leuk Lymphoma. 2003 Nov;44(11):1997-2000. doi: 10.1080/1042819031000112575.
Intravascular lymphoma (IVL) is an extremely rare form of extra-nodal non-Hodgkins lymphoma characterised by the proliferation of neoplastic lymphocytes within the lumina of small arteries, veins and capillaries. The great majority of reported cases appear to be of B cell lineage. There is a wide variation in clinical presentation, and multiple organs are usually affected. We report a case of a 67-year-old man who presented with constitutional symptoms and neurological deficit and was diagnosed following bone-marrow trephine. His disease responded to polychemotherapy treatment but he died 15 months after diagnosis. This case in unusual in that it is generally felt that bone marrow is relatively spared until late in the disease and is often not clearly demonstrable histologically. In addition, this case supports the limited data that responses can be obtained following polychemotherapy treatment, although the prognosis remains generally poor.
血管内淋巴瘤（IVL）是一种极其罕见的结外非霍奇金淋巴瘤，其特征是肿瘤性淋巴细胞在小动脉、静脉和毛细血管腔内增殖。绝大多数报告病例似乎为B细胞谱系。临床表现差异很大，多个器官通常会受到影响。我们报告一例67岁男性病例，该患者出现全身症状和神经功能缺损，经骨髓环钻活检后确诊。他的疾病对多药化疗有反应，但在诊断后15个月死亡。该病例不同寻常之处在于，一般认为骨髓在疾病晚期之前相对较少受累，且在组织学上往往难以明确显示。此外，该病例支持了有限的数据，即多药化疗后可获得反应，尽管总体预后仍然较差。
