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肺血管内淋巴瘤的临床及病理特征
Clinical and Pathological Features of Pulmonary Intravascular Lymphoma
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摘要:目的 探讨肺血管内淋巴瘤的临床及病理特征。方法 回顾性观察及总结了2008年3月至2011年6月北京协和医院经肺活检病理诊断的5例肺血管内淋巴瘤患者的临床、胸部电子计算机断层摄影(computed tomography, CT)及病理资料, 并复习文献。活检标本经10%中性福尔马林固定, 常规石蜡切片, HE及免疫组化染色。结果 5例肺血管内淋巴瘤患者中, 男3例, 女2例; 年龄36~59岁, 中位年龄45岁。临床症状主要为发热(5/5)、体重减轻(5/5)、咳嗽(4/5)、乏力(3/5)和气短(2/5)等; 5例患者血清乳酸脱氢酶均有不同程度升高(316~1025 U/L)。肺功能以弥漫性功能障碍为主。肺部CT:3例表现为双肺多发磨玻璃及实变影, 1例为多发结节影、胸膜下楔形实变影及支气管血管束增粗, 1例表现为右肺下叶实变影。病理组织学:肺血管内淋巴瘤主要表现为单个或小簇淋巴瘤细胞分布于狭小的肺泡间隔毛细血管腔内, 保留肺泡结构, 伴有肺泡上皮增生, 其病变微小, 易漏诊。1例肿瘤细胞形成瘤栓伴肺梗死。免疫组化显示3例为B细胞性, 2例为T细胞性。随访20 d~6个月(平均3.17个月), 3例B细胞性血管内淋巴瘤患者均行R-CHOP方案化疗, 2例化疗后病情平稳存活, 1例化疗6程后病情进展, 更换Hyper-CVAD方案化疗后病情平稳存活; 2例T细胞性血管内淋巴瘤患者, 1例失访, 1例经CHOP方案化疗效果不佳, 于诊断后20 d死于消化道出血和呼吸衰竭。结论 肺血管内淋巴瘤非常少见, 常见于中老年人, 其临床症状缺乏特异性, 胸部CT常表现为弥漫性间质性病变。病理组织学显示, 淋巴瘤细胞主要分布于狭小的肺泡间隔毛细血管腔内, 其病变微小、易漏诊, 免疫组化有助于其诊断。Abstract:Objective To investigate the clinical and pathological features of pulmonary intravascular lymphoma (IVL).Methods The clinical, radiographic, and pathological features of five cases of pulmonary IVL diagnosed by lung biopsy from March 2008 to June 2011 were retrospectively analyzed, and similar cases reported in English literatures were reviewed.Results These 5 patients (3 males and 2 females) aged 36 to 59 years, with a median age of 45 years. The chief complaints included fever (5/5), weight loss (5/5), cough (4/5), fatigue (3/5), and dyspnea (2/5). Pulmonary function tests mainly showed some degree of decreased diffusing capacity. All the five cases had high serum lactate dehydrogenase (range:316-1025 U/L). Chest CT showed diffuse ground-glass opacity (n=3), bilateral multiple nodular density with subpleural wedgeshaped density and thickening of bronchial wall (n=1), and consolidations in the right lower lobe (n=1). On histopathology, single or clustered lymphoma cells were seen within the lumens of capillaries of the alveolar septum, with the alveolar structure remained; the lesion was small and could not be easily identified. Pulmonary infarction caused by tumor thrombus was identified in one case. Immunophenotyping showed B cell lineage in 3 cases and T cell lineage in 2 cases. During the follow-up period (range:0.67-6 months; mean:3.17 months), 3 patients with B cell pulmonary IVL received R-CHOP chemotherapy and their diseases were improved and remained stable. One patient with T cell pulmonary IVL received CHOP chemotherapy but died due to gastrointestinal bleeding and respiratory failure 20 days after diagnosis. One patient with T cell pulmonary IVL was lost to follow-up.Conclusions Pulmonary IVL is a rare malignant disease that mainly seen in the elderly. Its clinical symptoms are not specific. Chest CT usually reveals diffuse interstitial changes, which should be carefully differentiated from interstitial pneumonia, pulmonary infection, and lung metastatic cancer. It can be pathologically diagnosed through transbronchial lung biopsy or surgical lung biopsy.
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