Allen James, Shaikh Anam, Laurent-Ariot Karrah, Merola Matthew
Internal Medicine, St. John's Episcopal Hospital, New York, USA.
Psychiatry, St. John's Episcopal Hospital, New York, USA.
Cureus. 2022 Jun 14;14(6):e25920. doi: 10.7759/cureus.25920. eCollection 2022 Jun.
Intravascular large B-cell lymphoma (IVLBCL) is a subtype of extranodal lymphoma that characteristically contains malignant lymphocytes within blood vessels. The clinical presentation of IVLBCL has high variability. In our case, the patient's initial presentation involved bilateral ptosis, restricted extraocular movements, periorbital pain, and bitemporal headache. The patient denied the classic "B symptoms" such as fever, night sweats, or weight loss. The patient also denied a family history of malignancy. Initial imaging studies were unremarkable, making diagnosis particularly challenging. Ultimately, functional endoscopic sinus surgery was performed. Pathological examination of the intraoperative specimen revealed a CD5+ large B-cell lymphoma within the vessels involving the left ethmoid sinus, respiratory mucosa, and nasal septum. The patient underwent steroid therapy prior to diagnosis, which led to rapid improvement in headache and mild improvement in extraocular function and ptosis. Following diagnosis, the patient underwent chemotherapy with supportive medications. Our case report may be considered a reference for cases presenting with extensive bilateral extraocular muscle deficits and levator palpebrae dysfunction in the absence of notable initial imaging findings, "B symptoms," or positive family history. The teaching point from this case is to demonstrate the difficulty of diagnosis and our train of thought in investigating an abnormal presentation with no clearly identifiable etiology following initial diagnostic workup and treatment.
血管内大B细胞淋巴瘤(IVLBCL)是一种结外淋巴瘤亚型,其特征是血管内含有恶性淋巴细胞。IVLBCL的临床表现具有高度变异性。在我们的病例中,患者最初的表现包括双侧上睑下垂、眼球运动受限、眶周疼痛和双侧颞部头痛。患者否认有发热、盗汗或体重减轻等典型的“B症状”。患者也否认有恶性肿瘤家族史。最初的影像学检查无明显异常,这使得诊断极具挑战性。最终,进行了功能性内镜鼻窦手术。术中标本的病理检查显示,血管内存在CD5+大B细胞淋巴瘤,累及左侧筛窦、呼吸黏膜和鼻中隔。患者在诊断前接受了类固醇治疗,这使得头痛迅速改善,眼外肌功能和上睑下垂也有轻度改善。确诊后,患者接受了化疗及支持性药物治疗。我们的病例报告可作为参考,用于那些在没有明显的初始影像学表现、“B症状”或阳性家族史的情况下,出现广泛双侧眼外肌功能障碍和提上睑肌功能障碍的病例。这个病例的教学要点在于展示诊断的困难以及我们在对初始诊断检查和治疗后病因不明的异常表现进行调查时的思路。