Hamada Takashi, Takahashi Hiromichi, Nakagawa Masaru, Nukariya Hironao, Ito Shun, Endo Toshihide, Kurihara Kazuya, Koike Takashi, Iizuka Kazuhide, Ohtake Shimon, Ichinohe Takashi, Maebayashi Toshiya, Miura Katsuhiro, Hatta Yoshihiro, Nakamura Hideki
Division of Hematology and Rheumatology, Department of Medicine, Nihon University School of Medicine, Tokyo, Japan.
Division of Laboratory Medicine, Department of Pathology and Microbiology, Nihon University School of Medicine, Tokyo, Japan.
Case Rep Oncol. 2023 May 22;16(1):338-346. doi: 10.1159/000530265. eCollection 2023 Jan-Dec.
Pulmonary veno-occlusive disease (PVOD) is an extremely rare condition in oncology practice. Although PVOD is clinically similar to pulmonary arterial hypertension, the conditions differ in terms of pathophysiology, management, and prognosis. This report discusses the case of a 47-year-old woman who developed dyspnea and fatigue after high-dose cyclophosphamide chemotherapy and autologous hematopoietic stem cell transplantation for relapsed lymphoma. The patient exhibited tachycardia, tachypnea, and hypotension, but other findings in the physical examination were unremarkable. The imaging studies showed no evidence of pulmonary embolism, but multiple ground-glass opacities and bilateral pleural effusions were observed on chest high-resolution computed tomography scans. In the right heart catheterization study, the mean pulmonary artery pressure and pulmonary vascular resistance were 35 mm Hg and 5.93 Wood units, respectively, with a normal pulmonary capillary wedge pressure of 10 mm Hg. Pulmonary function tests revealed a remarkable reduction in the percentage predicted value of diffusing capacity of the lungs for carbon monoxide to 31%. Lymphoma progression, collagen diseases, infectious diseases such as human immunodeficiency virus or parasitic infections, portal hypertension, and congenital heart disease were carefully excluded as these are also capable of causing pulmonary arterial hypertension. Thereafter, we reached a final diagnosis of PVOD. The patient was treated with supplemental oxygen and a diuretic during 1 month of hospitalization, which relieved her right heart overload symptoms. Herein, we present the patient's clinical course and diagnostic workup because misdiagnosis or inappropriate treatment can lead to unfavorable outcomes in patients with PVOD.
肺静脉闭塞性疾病（PVOD）在肿瘤学实践中是一种极其罕见的病症。尽管PVOD在临床上与肺动脉高压相似，但在病理生理学、治疗和预后方面存在差异。本报告讨论了一名47岁女性的病例，该患者在接受高剂量环磷酰胺化疗及自体造血干细胞移植治疗复发性淋巴瘤后出现呼吸困难和疲劳。患者表现出心动过速、呼吸急促和低血压，但体格检查的其他结果并无异常。影像学检查未发现肺栓塞的证据，但胸部高分辨率计算机断层扫描显示有多处磨玻璃影和双侧胸腔积液。在右心导管检查中，平均肺动脉压和肺血管阻力分别为35 mmHg和5.93 Wood单位，肺毛细血管楔压正常，为10 mmHg。肺功能测试显示，肺一氧化碳弥散能力预测值百分比显著降低至31%。仔细排除了淋巴瘤进展、胶原疾病、人类免疫缺陷病毒等传染病或寄生虫感染、门静脉高压和先天性心脏病，因为这些也可能导致肺动脉高压。此后，我们最终诊断为PVOD。患者在住院1个月期间接受了吸氧和利尿剂治疗，这缓解了她的右心负荷过重症状。在此，我们介绍该患者的临床病程和诊断检查情况，因为误诊或不恰当的治疗可能导致PVOD患者出现不良后果。
