van Paassen J, Jonkers G J P M
Rijnland Ziekenhuis, locatie St. Elisabeth, afd. Interne Geneeskunde, Leiderdorp.
Ned Tijdschr Geneeskd. 2005 May 14;149(20):1118-22.
A 69-year-old man presented with dyspnoea, cough and diffuse interstitial changes and ground-glass effect on a CT-scan. After 5 months the patient was diagnosed with pulmonary intravascular lymphomatosis. A histologic diagnosis was made by a video-assisted thoracoscopic surgical biopsy. Treatment with combination chemotherapy, i.e. 8 cycles of cyclophosphamide-doxorubicin-vincristine-prednisone (CHOP), resulted in a complete remission and a disease-free survival of 5 years at the last follow-up. Intravascular lymphomatosis is a rare but curable cause of interstitial lung disease. Intravascular lymphomatosis is an uncommon type of non-Hodgkin's lymphoma, characterized by proliferation of neoplastic lymphoid cells within the lumen of small blood vessels, resulting in thrombotic and ischaemic complications in multiple organ systems. Primary pulmonary presentation is even more uncommon. It is important to consider intravascular lymphomatosis in the differential diagnosis of unexplained interstitial lung disease, since early diagnosis and treatment may lead to complete remission and long-term survival.
一名69岁男性患者出现呼吸困难、咳嗽，CT扫描显示弥漫性间质改变及磨玻璃影。5个月后，该患者被诊断为肺血管内淋巴瘤。通过电视辅助胸腔镜手术活检进行了组织学诊断。采用环磷酰胺-阿霉素-长春新碱-泼尼松（CHOP）联合化疗8个周期，最后一次随访时达到完全缓解，无病生存期达5年。血管内淋巴瘤是间质性肺疾病的一种罕见但可治愈的病因。血管内淋巴瘤是一种不常见的非霍奇金淋巴瘤类型，其特征为肿瘤性淋巴细胞在小血管腔内增殖，导致多器官系统出现血栓形成和缺血性并发症。原发性肺部表现更为罕见。在不明原因间质性肺疾病的鉴别诊断中考虑血管内淋巴瘤很重要，因为早期诊断和治疗可能导致完全缓解和长期生存。
