Department of Hematology, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan.
Department of Pathology, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan.
J Clin Exp Hematop. 2022 Mar 9;62(1):41-45. doi: 10.3960/jslrt.21013. Epub 2021 Nov 26.
Intravascular large B-cell lymphoma (IVLBCL) is a rare lymphoma characterized by the selective growth of lymphoma cells within the lumen of vessels. We describe the case of a 69-year-old male who presented with marked pain in the left facial region. Gadolinium-enhanced magnetic resonance imaging revealed a swollen left trigeminal nerve (TN) and positron emission tomography/computed tomography demonstrated fluorodeoxyglucose-only uptake at the same site. The patient had high serum lactate dehydrogenase and soluble interleukin-2 receptor levels. As random skin biopsy and bone marrow biopsy detected no abnormal pathogenesis, open biopsy of the TN was performed, revealing diffuse large B-cell lymphoma (DLBCL). However, ground glass opacities rapidly developed in both lung fields with severe respiratory failure. The patient died of progressive disease before the initiation of chemotherapy. Postmortem examination revealed widespread lymphoma cells in the lumen of vessels in multiple organs, including the lungs, excluding the bone marrow and skin. Lymphoma cells formed a mass in the TN and left lumbar plexus. A diagnosis of IVLBCL was made based on the postmortem pathological analysis. DLBCL of abnormal sites, such as the peripheral nervous system, should be considered in cases of IVLBCL as a differential diagnosis.
血管内大 B 细胞淋巴瘤(IVLBCL)是一种罕见的淋巴瘤,其特征是淋巴瘤细胞选择性地在血管腔内生长。我们描述了一例 69 岁男性患者,其左面部区域出现明显疼痛。钆增强磁共振成像显示左侧三叉神经(TN)肿胀,正电子发射断层扫描/计算机断层扫描显示同一部位仅摄取氟脱氧葡萄糖。患者血清乳酸脱氢酶和可溶性白细胞介素-2 受体水平升高。由于随机皮肤活检和骨髓活检未发现异常发病机制,对 TN 进行了开放性活检,结果显示弥漫性大 B 细胞淋巴瘤(DLBCL)。然而,双肺野迅速出现磨玻璃样混浊,伴有严重呼吸衰竭。在开始化疗之前,患者因疾病进展而死亡。尸检显示多个器官的血管腔内有广泛的淋巴瘤细胞,包括肺,但骨髓和皮肤除外。淋巴瘤细胞在 TN 和左腰丛中形成肿块。根据尸检病理分析诊断为 IVLBCL。在 IVLBCL 病例中,应考虑包括周围神经系统在内的异常部位的 DLBCL 作为鉴别诊断。