Suh C H, Kim S K, Shin D H, Chung K Y, Kim S K
Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
J Korean Med Sci. 1997 Oct;12(5):457-60. doi: 10.3346/jkms.1997.12.5.457.
Intravascular lymphomatosis (IL) is a rare and generally fatal disease characterized by proliferation of large lymphoma cells almost exclusively within the lumen of small blood vessels. The skin and central nervous systems are typically affected, but involvement of other organs, such as lung, has been described. Predominant lung involvement without cutaneous and neurologic manifestation is very rare and difficult to diagnose. Originally considered as an endothelial disorder, IL has recently been reclassified as lymphoma. Most of the cases reported are of B cell lineage with a few cases of T cell type. We describe a case of the T-cell type IL manifested clinically as an interstitial lung disease without involvement of skin and central nervous systems. Immunohistochemical studies showed the T-cell nature of the neoplastic cells in open lung biopsy sample.
血管内淋巴瘤（IL）是一种罕见且通常致命的疾病，其特征是大淋巴瘤细胞几乎仅在小血管腔内增殖。皮肤和中枢神经系统通常会受到影响，但也有其他器官受累的描述，如肺。以肺部为主且无皮肤和神经表现的情况非常罕见且难以诊断。IL最初被认为是一种内皮疾病，最近已被重新归类为淋巴瘤。报道的大多数病例为B细胞谱系，少数为T细胞类型。我们描述了一例T细胞型IL，临床上表现为间质性肺病，无皮肤和中枢神经系统受累。免疫组织化学研究显示开胸肺活检样本中肿瘤细胞具有T细胞性质。
