Demirer T, Dail D H, Aboulafia D M
Division of Oncology, Virginia Mason Medical Center, Seattle, WA 98111.
Cancer. 1994 Mar 15;73(6):1738-45. doi: 10.1002/1097-0142(19940315)73:6<1738::aid-cncr2820730631>3.0.co;2-u.
Intravascular lymphoma (IVL) is an uncommon malignancy defined pathologically by neoplastic proliferation of lymphoid cells within the lumens of capillaries, small veins, and arteries, with little or no adjacent parenchymal involvement. The nature of IVL has been the subject of considerable controversy. Recent immunohistochemical studies have demonstrated clearly that the tumors bear the immunophenotype of neoplastic lymphoid cells of either B-cell or T-cell lineage. IVL commonly affects the central nervous system, resulting in progressive dementia and multiple neurologic deficits; skin is the second most common site of involvement, in the form of cutaneous plaques and nodules.
In a retrospective review of all cases of non-Hodgkin's lymphoma seen at our institution, four cases of IVL were identified. Case 1 was fixed in methacarin, and Cases 2-4 were fixed in 10% formalin. Standard fixation, tissue processing, sectioning, and hematoxylin and eosin staining were used. Immunophenotypic studies were performed using a modified avidin-biotin complex technique. The specimen in Case 1 was stained by Giemsa stain using standard techniques.
Four cases of IVL are presented. One patient experienced hypoxemia and fevers of unknown origin; the second, dementia and a vasculitislike illness; the third rapidly progressive dementia, nonlocalizing neurologic deficits, and panhypopituitarism; the fourth Kaposi-like skin lesions. Case 1 relapsed shortly after completing chemotherapy. Salvage chemotherapy has yielded good initial response. Autopsy findings in cases 2, 3 and 4 confirmed IVL in many vessels, including the brain, lung, liver, heart, gastrointestinal tract, glomerulus, central nervous system, and skin. Malignant lymphoid cells marked as B-cells in all cases.
Unusual and interesting clinical presentations may occur in patients with IVL. The medical literature indicates that few cases are diagnosed ante mortem. Although isolated patients may respond favorably to chemotherapy, most will deteriorate rapidly and the diagnosis of IVL not contemplated until necropsy.
血管内淋巴瘤（IVL）是一种罕见的恶性肿瘤，其病理定义为淋巴样细胞在毛细血管、小静脉和动脉管腔内的肿瘤性增殖，几乎没有或没有相邻实质受累。IVL的性质一直是相当有争议的话题。最近的免疫组织化学研究清楚地表明，这些肿瘤具有B细胞或T细胞系肿瘤性淋巴样细胞的免疫表型。IVL通常累及中枢神经系统，导致进行性痴呆和多种神经功能缺损；皮肤是第二常见的受累部位，表现为皮肤斑块和结节。
在对我院所见的所有非霍奇金淋巴瘤病例进行回顾性研究时，确定了4例IVL。病例1用美他卡林固定，病例2 - 4用10%福尔马林固定。采用标准固定、组织处理、切片及苏木精和伊红染色。免疫表型研究采用改良抗生物素蛋白 - 生物素复合物技术。病例1的标本采用标准技术进行吉姆萨染色。
报告了4例IVL。1例患者出现低氧血症和不明原因发热；第2例有痴呆和血管炎样疾病；第3例有快速进展性痴呆、无定位神经功能缺损和全垂体功能减退；第4例有卡波西样皮肤病变。病例1在完成化疗后不久复发。挽救性化疗取得了良好的初始反应。病例2、3和4的尸检结果证实，在包括脑、肺、肝、心、胃肠道、肾小球、中枢神经系统和皮肤在内的许多血管中存在IVL。所有病例中的恶性淋巴样细胞均标记为B细胞。
IVL患者可能出现不寻常且有趣的临床表现。医学文献表明，生前诊断出的病例很少。虽然个别患者可能对化疗反应良好，但大多数患者病情会迅速恶化，直到尸检时才考虑诊断为IVL。
