Ponzoni Maurilio, Ferreri Andrés J M
Pathology Unit, San Raffaele H Scientific Institute, Milan, Italy.
Hematol Oncol. 2006 Sep;24(3):105-12. doi: 10.1002/hon.776.
Intravascular lymphoma (IVL) is an extremely rare form of non-Hodgkin lymphoma characterized by almost exclusive growth of neoplastic lymphocytes within blood vessel lumen. IVL is morphologically characterized in most instances by large cells with B-cell lineage. IVL is an aggressive and usually disseminated disease that predominantly affects elderly patients, resulting in poor PS, B-symptoms, anemia, and high lactate dehydrogenase serum level. The brain and skin are the most commonly involved sites; nodal disease is rare. Survival after conventional chemotherapy is disappointing, with a relevant impact of diagnostic delay and lethal complications. Notwithstanding these results, IVL limited to the skin (cutaneous variant) is a favorable presentation with distinctive clinical characteristics. Moreover, differences in clinical presentation with Eastern Countries IVL cases, mostly associated with hemophagocytic syndrome, do exist. Intensive combinations containing drugs with higher central nervous system bioavailability are needed in cases with brain involvement; the role of high-dose chemotherapy with autologous stem cell transplantation should be investigated in younger patients with unfavorable features. The present review will discuss the most recent acquisitions related either to diagnosis and immunophenotypic/biologic characteristics as well as clinical/therapeutic issues of IVL.
血管内淋巴瘤（IVL）是一种极其罕见的非霍奇金淋巴瘤，其特征是肿瘤性淋巴细胞几乎仅在血管腔内生长。在大多数情况下，IVL在形态学上的特征是具有B细胞系的大细胞。IVL是一种侵袭性疾病，通常会播散，主要影响老年患者，导致较差的体能状态、B症状、贫血和血清乳酸脱氢酶水平升高。脑和皮肤是最常受累的部位；淋巴结受累罕见。传统化疗后的生存率令人失望，诊断延迟和致命并发症有显著影响。尽管有这些结果，但局限于皮肤的IVL（皮肤型）是一种具有独特临床特征的良好表现形式。此外，与东方国家的IVL病例在临床表现上确实存在差异，这些病例大多与噬血细胞综合征相关。对于脑受累的病例，需要使用具有更高中枢神经系统生物利用度的药物进行强化联合治疗；对于具有不良特征较年轻的患者，应研究大剂量化疗联合自体干细胞移植的作用。本综述将讨论与IVL的诊断、免疫表型/生物学特征以及临床/治疗问题相关的最新进展。
