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寡分泌性多发性骨髓瘤:一种具有挑战性诊断的毁灭性表现。

Oligosecretory multiple myeloma: a devastating presentation of a difficult diagnosis.

机构信息

Internal Medicine Department, Hospital and University Centre of Coimbra, Coimbra, Portugal

Internal Medicine Department, Hospital and University Centre of Coimbra, Coimbra, Portugal.

出版信息

BMJ Case Rep. 2021 Apr 22;14(4):e240404. doi: 10.1136/bcr-2020-240404.

Abstract

A 57-year-old man with lumbar pain and fever was diagnosed with spondylodiscitis. Afterward, he acquired full paraplegia. Image studies showed a mass extending from D9 to the vertebral canal, plus numerous adjacent osteolytic lesions. Serum immunoelectrophoresis was normal, bone marrow had 0.5% of monoclonal plasmocytes, but D9's biopsy found a plasmacytoma. Despite bone marrow aspiration results, skeleton osteolytic lesions made multiple myeloma (MM) a more plausible diagnosis, later confirmed by the biopsy. The absence of classical MM findings, alongside a medullary compression syndrome, suggested an oligosecretory MM, which was proved by an altered FLC essay. This delayed diagnosis, with multiple diagnostic misguiding leads, also presents rare IgA and lambda chains production and normal levels of uninvolved immunoglobulins. Oligosecretory MM can lead to an inaccurate and delayed diagnosis, with devastating consequences to patient's morbidity and mortality. Therefore, FLC essay is essential in early assessment of potential MM patients.

摘要

一位 57 岁男性,腰痛伴发热,诊断为脊椎骨髓炎。之后,他完全截瘫。影像学研究显示从 D9 延伸到椎管的肿块,加上许多相邻的溶骨性病变。血清免疫电泳正常,骨髓有 0.5%的单克隆浆细胞,但 D9 的活检发现浆细胞瘤。尽管骨髓抽吸结果正常,但骨骼溶骨性病变使多发性骨髓瘤(MM)成为更合理的诊断,后来通过活检证实。缺乏典型 MM 表现,加上骨髓压迫综合征,提示为寡分泌型 MM,这通过游离轻链(FLC)检测得到证实。这种诊断延迟,有多个误导性的诊断线索,也表现出罕见的 IgA 和 lambda 链产生以及未受累免疫球蛋白的正常水平。寡分泌型 MM 可导致不准确和延迟的诊断,对患者的发病率和死亡率产生严重后果。因此,FLC 检测在潜在 MM 患者的早期评估中至关重要。

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本文引用的文献

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J Antimicrob Chemother. 2010 Nov;65 Suppl 3:iii11-24. doi: 10.1093/jac/dkq303.
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Current diagnosis and treatment of spondylodiscitis.目前对椎间盘炎的诊断和治疗。
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