Tsuji Mayoko, Kondo Mitsuko, Onizawa Fumi, Shishime Osamu, Muramatsu Soshi, Matsuo Yuka, Sakai Shuji, Tanaka Junji, Tagaya Etsuko
Department of Respiratory Medicine, Tokyo Women's Medical University, Tokyo, 162-8666, Japan.
Department of Diagnostic Imaging and Nuclear Medicine, Tokyo Women's Medical University, Tokyo, 162-8666, Japan.
Respir Med Case Rep. 2022 Mar 11;37:101625. doi: 10.1016/j.rmcr.2022.101625. eCollection 2022.
A 56-year-old man complained progressive dyspnea, fatigue and fever for one month. His chest CT exhibited faint ground-glass opacities, and the levels of serum LDH and soluble interleukin 2 receptor were markedly elevated. Positron emission tomography (PET) showed high uptake of 18-fluoro deoxy glucose (FDG) only on both lungs. We performed transbronchial lung biopsies (TBLB) for the diagnosis. After bronchoscopy, he had prolonged hypoxemia. Because defects of 99m-Technetium macroaggregated albumin (Tc-MAA) in pulmonary blood flow scintigraphy were consistent with the distribution of FDG uptake in PET, we speculated that the presence of intravascular lymphoma (IVL) cells in the capillaries might have behaved like tumor embolism. We started rescue by prednisolone based on treatment of lymphoma. As a result, his hypoxemia was gradually improved. Histological findings in TBLB specimen showed that CD20CD79Bcl-2c-myc lymphoma cells were localized to small vessel lumina in alveoli and bronchioles, and he was definitely diagnosed with lung intravascular large B cell lymphoma (IVLBCL). He was treated with complete cyclophosphamide, doxorubicin, vincristine, and prednisolone with rituximab (R-CHOP) in combination with intrathecal methotrexate injection. After eight cycles of R-CHOP and three times of intrathecal methotrexate, FDG uptake of PET on both lungs completely disappeared, achieving complete metabolic remission. We experienced a rare case of lung IVLBCL developed with respiratory failure successfully rescued by prednisolone prior to definite diagnosis.
一名56岁男性，出现进行性呼吸困难、乏力和发热1个月。其胸部CT显示有淡薄的磨玻璃影，血清乳酸脱氢酶（LDH）水平和可溶性白细胞介素2受体水平显著升高。正电子发射断层扫描（PET）显示仅双肺18-氟脱氧葡萄糖（FDG）摄取增高。为明确诊断，我们进行了经支气管肺活检（TBLB）。支气管镜检查后，他出现了长时间的低氧血症。由于肺血流灌注显像中99m-锝标记的大颗粒白蛋白（Tc-MAA）缺损与PET中FDG摄取分布一致，我们推测毛细血管内血管内淋巴瘤（IVL）细胞的存在可能表现为肿瘤栓塞。我们基于淋巴瘤的治疗方案开始使用泼尼松龙进行抢救。结果，他的低氧血症逐渐改善。TBLB标本的组织学检查结果显示，CD20、CD79、Bcl-2、c-myc淋巴瘤细胞定位于肺泡和细支气管的小血管腔内，他被明确诊断为肺血管内大B细胞淋巴瘤（IVLBCL）。他接受了环磷酰胺、多柔比星、长春新碱和泼尼松龙联合利妥昔单抗（R-CHOP）并鞘内注射甲氨蝶呤的完整治疗方案。经过8个周期的R-CHOP治疗和3次鞘内注射甲氨蝶呤后，PET显示双肺的FDG摄取完全消失，实现了完全代谢缓解。我们遇到了一例罕见的肺IVLBCL病例，在明确诊断前因呼吸衰竭而成功地通过泼尼松龙抢救过来。
