背景:带有干扰素调节因子4(IRF4)重排(LBCL-IRF4)的大B细胞淋巴瘤(LBCL)是LBCL的一种罕见亚型,Waldeyer的戒指和脖子的患病率很高,头部和胃肠道淋巴结。
方法:报道1例2个月临床症状为鼻塞和面部肿胀的患者。鼻内窥镜检查显示下鼻道有肿瘤。CT和增强MRI均显示软组织占据鼻泪管,骨骼破坏,并延伸到左侧鼻腔和左侧泪腺区。然后,对下鼻道的肿瘤进行了活检。
结果:HE染色结果显示肿瘤细胞呈现弥漫性生长模式,丰富的细胞质,液泡形状,轻微染色的细胞核,和不规则的核膜。免疫组化染色结果显示MUM1(+),Bcl6(+),CD20(+),CD79α(+),和CD10(+)。FISH分析检测到IRF4重排阳性。患者诊断为LBCL-IRF4。患者接受了四个周期的R-CHOP和两次利妥昔单抗的治疗,随访2年,终于完全缓解了.
结论:第一次,我们总结了影像学和病理学特征,药物治疗,LBCL-IRF4在鼻泪管中的疗效。
BACKGROUND: Large B-cell lymphoma (LBCL) with interferon regulatory factor 4 (IRF4) rearrangement (LBCL-IRF4) is a rare subtype of LBCL, with a high prevalence in Waldeyer\'s ring as well as the neck, head and gastrointestinal lymph nodes.
METHODS: A patient with 2-month clinical symptoms of nasal obstruction and facial swelling was reported in this short review. A nasal endoscopy examination revealed a neoplasm in the inferior nasal meatus. Both CT and enhanced MRI showed that a soft tissue occupied the nasolacrimal duct, with bone destruction, and extended into the left nasal cavity and left lacrimal gland area. Then, a biopsy of the neoplasm in the inferior nasal meatus was performed.
RESULTS: HE staining results showed that neoplastic cells presented diffuse growth patterns, abundant cytoplasm, vacuole shape, lightly stained nuclei, and irregular nuclear membrane. Immunohistochemistry staining results revealed MUM1(+), Bcl6(+), CD20(+), CD79α(+), and CD10(+). FISH analyses detected positive IRF4 rearrangement. LBCL-IRF4 was diagnosed in the patient. The patient received treatment with four cycles of R-CHOP and two times of rituximab, followed up for 2 years, and finally got complete remission.
CONCLUSIONS: For the first time, we summarize the imaging and pathological features, drug treatment, and curative effect of LBCL-IRF4 in the nasolacrimal duct.