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多发性骨髓瘤伴单侧突然视力丧失的罕见表现:一例报告。

An unusual presentation of multiple myeloma with unilateral sudden vision loss: A case report.

作者信息

Wu Pei-Wen, Lee Ta-Jen, Chen Jim-Ray, Huang Chien-Chia

机构信息

Department of Otolaryngology-Head and Neck Surgery, Chang Gung Memorial Hospital and Chang Gung University, Keelung Division of Rhinology, Department of Otolaryngology, Chang Gung Memorial Hospital and Chang Gung University, Taoyuan Department of Pathology, Chang Gung Memorial Hospital and Chang Gung University, Keelung Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung University, Taoyuan, Taiwan.

出版信息

Medicine (Baltimore). 2017 Jun;96(25):e7277. doi: 10.1097/MD.0000000000007277.

Abstract

RATIONALE

Plasma cell neoplasms are categorized by neoplastic proliferation of a single clone of plasma cells which produce a monoclonal immunoglobulin. Plasma cell neoplasms can present as a solitary plasmacytoma or as multiple myeloma. Both of them can progress to multiple myeloma. Once a diagnosis of plasmacytoma has been made, thorough examinations should be carried out for identifying the disease entity.

PATIENT CONCERNS AND DIAGNOSES

Herein, we describe an extraordinary rare case of multiple myeloma with initial presentation of a left sphenoid neoplasm resulting in left-sided headache and rapid deterioration of visual acuity. Histo-pathologic analysis revealed a plasma cell neoplasm with positive immunostaining for cluster of differentiation (CD)138, CD79a, and kappa light chain of immunoglobulin. A bone marrow aspiration was then performed, and the diagnosis of multiple myeloma was then confirmed.

INTERVENTIONS AND OUTCOMES

After investigative workup, our patient received chemotherapy, localized radiotherapy, and autologous stem cell transplantation. Her visual acuity recovered to the baseline and she sustained a partial response without subjective discomfort.

LESSONS

Extramedullary plasmacytoma is an interesting but infrequent presentation of multiple myeloma. Moreover, involvement of the sphenoid sinus in multiple myeloma resulting in extrinsic optic nerve compression is extremely rare. Clinicians should consider plasmacytoma as a diagnostic possibility when presented with cases of solitary sphenoid neoplasm and rapid progression of clinical course.

摘要

原理

浆细胞肿瘤是由产生单克隆免疫球蛋白的单个浆细胞克隆性增殖所分类的。浆细胞肿瘤可表现为孤立性浆细胞瘤或多发性骨髓瘤。两者都可进展为多发性骨髓瘤。一旦做出浆细胞瘤的诊断,就应进行全面检查以确定疾病实体。

患者关注与诊断

在此,我们描述了一例极其罕见的多发性骨髓瘤病例,最初表现为左侧蝶骨肿瘤,导致左侧头痛和视力迅速恶化。组织病理学分析显示为浆细胞肿瘤,免疫组化显示分化簇(CD)138、CD79a和免疫球蛋白κ轻链呈阳性。随后进行了骨髓穿刺,确诊为多发性骨髓瘤。

干预措施与结果

经过检查后,我们的患者接受了化疗、局部放疗和自体干细胞移植。她的视力恢复到基线水平,维持了部分缓解且无主观不适。

经验教训

髓外浆细胞瘤是多发性骨髓瘤一种有趣但不常见的表现形式。此外,多发性骨髓瘤累及蝶窦导致视神经受压极为罕见。当遇到孤立性蝶骨肿瘤且临床病程快速进展的病例时,临床医生应考虑浆细胞瘤作为一种诊断可能性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a952/5484246/352b18878eff/medi-96-e7277-g001.jpg

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