Yamagata Toshiyuki, Okamoto Yukiharu, Ota Kiichiro, Katayama Norifumi, Tsuda Tadaaki, Yukawa Susumu
Division of Clinical Oncology and Palliative Medicine, Wakayama Medical University, Wakayama, Japan.
Respiration. 2003 Jul-Aug;70(4):414-8. doi: 10.1159/000072907.
Intravascular lymphomatosis with primary pulmonary lesion is an extremely rare disease. Although the major clinical symptoms include fever, cough, dyspnea and loss of body weight, these are not diagnostic. Chest radiograph findings are also nonspecific and include bilateral reticular shadow, reticulonodular shadow, ground-glass opacity or wedge-shaped subpleural opacities. Therefore, the antemortem diagnosis is relatively difficult. It is considered that intravascular lymphomatosis is a high-grade malignant lymphoma. However, it has been shown recently that a good response and long-term survival may possibly be obtained through systemic combination chemotherapy. We report a case of intravascular lymphomatosis with primary pulmonary lesion where an early diagnosis was obtained through thoracoscopic lung biopsy and subsequent systemic chemotherapy proved to be quite effective. Because the clinical symptoms or chest radiograph findings are usually nonspecific, it was thought that thoracoscopic lung biopsy could be a useful procedure for early and reliable diagnosis of primary pulmonary intravascular lymphomatosis and that it might contribute to an improved prognosis.
原发性肺部病变的血管内淋巴瘤是一种极其罕见的疾病。虽然主要临床症状包括发热、咳嗽、呼吸困难和体重减轻，但这些均无诊断意义。胸部X线片表现也不具有特异性，包括双侧网状阴影、网状结节阴影、磨玻璃样混浊或楔形胸膜下混浊。因此，生前诊断相对困难。血管内淋巴瘤被认为是一种高度恶性淋巴瘤。然而，最近研究表明，通过全身联合化疗可能获得良好疗效和长期生存。我们报告一例原发性肺部病变的血管内淋巴瘤病例，该病例通过胸腔镜肺活检获得早期诊断，随后的全身化疗证明非常有效。由于临床症状或胸部X线片表现通常不具有特异性，因此认为胸腔镜肺活检可能是早期、可靠诊断原发性肺血管内淋巴瘤的有用方法，且可能有助于改善预后。
