METHODS: We present a case of a 57 years-old male who presented to the emergency department with a 5-day history of painful bilateral digital edema and color change episodes (from pallor to cyanosis). Physical examination revealed erythrocyanosis in the distal extremities, prompting consideration of secondary Raynaud syndrome. Despite medical therapy, progressive digital ischemia led to multiple finger amputations. During etiological investigation, anticoagulation tests and autoimmune analysis yielded negative results. A transesophageal echocardiogram was performed, revealing an irregular hyperechogenic mass on the anterior leaflet of the mitral valve without valve dysfunction, and a thoracic computed tomography scan with contrast showed an enlarged right paratracheal lymph node. Histopathological analysis from a transthoracic needle biopsy of the paratracheal lymph node revealed diffuse large B-cell lymphoma. The patient underwent aggressive R-CHOP chemotherapy, achieving a favorable complete response.
CONCLUSIONS: This is a particular case involving the occurrence of NBTE and Raynaud phenomenon as the initial paraneoplastic manifestations in a previously healthy young man. Reports of NBTE associated with lymphoproliferative conditions are quite rare, with fewer than ten cases described in the literature. To our knowledge, this is the first case of NBTE specifically associated with diffuse large B-cell lymphoma.
方法:我们介绍了一例57岁男性患者,他在急诊科就诊,有5天的双侧数字水肿和颜色变化发作(从苍白到发紫)的病史。体格检查显示四肢远端有红细胞增多症,提示考虑继发性雷诺综合征。尽管有药物治疗,进行性手指缺血导致多个手指截肢。在病因调查期间,抗凝试验和自身免疫分析结果为阴性.进行了经食管超声心动图检查,显示二尖瓣前小叶上有不规则的高回声肿块,无瓣膜功能障碍,胸部计算机断层扫描增强扫描显示右气管旁淋巴结肿大。气管旁淋巴结经胸穿刺活检的组织病理学分析显示弥漫性大B细胞淋巴瘤。患者接受了积极的R-CHOP化疗,实现有利的完整响应。
结论:这是一个特殊情况,涉及NBTE和雷诺现象的发生,作为先前健康的年轻人的最初副肿瘤表现。NBTE与淋巴组织增生相关的报道非常罕见,文献中描述的病例不到10例。据我们所知,这是首例与弥漫性大B细胞淋巴瘤特异性相关的NBTE病例.