Ko Y H, Han J H, Go J H, Kim D S, Kwon O J, Yang W I, Shin D H, Ree H J
Department of Diagnostic Pathology and Internal Medicine, Samsung Medical Centre, Sungkyunkwan University School of Medicine, Seoul, Korea.
Histopathology. 1997 Dec;31(6):555-62. doi: 10.1046/j.1365-2559.1997.3310898.x.
Intravascular lymphomatosis is an uncommon type of non-Hodgkin's lymphoma characterized by intravascular proliferation of neoplastic lymphoid cells. Although the tumour is basically a systemic disease, eventually involving multiple organs, primary presentation in the lung is rare.
We describe the clinicopathological features of two patients with intravascular lymphomatosis presenting in the lung. One patient complained of fever, headache and chest pain; the other, of dyspnoea on exertion and headache. Both patients showed reticulonodular density on chest radiography and decreased diffusion capacity. Lung biopsy showed features characteristic of intravascular lymphomatosis. Malignant lymphoid cells were CD30 positive T-cells of anaplastic large cell type in one patient and B-cells of large cell type in the other. There was a poor response to chemotherapy and both patients died of the disease within 3 months of diagnosis.
These cases and 10 previous reports illustrate the need to include intravascular lymphomatosis in the differential diagnosis of interstitial lung disease.
血管内淋巴瘤是一种罕见的非霍奇金淋巴瘤，其特征为肿瘤性淋巴样细胞的血管内增殖。尽管该肿瘤本质上是一种全身性疾病，最终会累及多个器官，但以肺部为首发表现的情况罕见。
我们描述了两例以肺部表现的血管内淋巴瘤患者的临床病理特征。一名患者主诉发热、头痛和胸痛；另一名患者主诉劳力性呼吸困难和头痛。两名患者胸部X线检查均显示网状结节状密度影，弥散功能降低。肺活检显示血管内淋巴瘤的特征性表现。一名患者的恶性淋巴样细胞为间变性大细胞型CD30阳性T细胞，另一名患者为大细胞型B细胞。化疗反应不佳，两名患者均在诊断后3个月内死于该病。
这些病例以及之前的10份报告表明，在间质性肺疾病的鉴别诊断中需要考虑血管内淋巴瘤。
