轻链骨髓瘤沉淀性冷球蛋白性血管炎。

IF 0.9 Q3 MEDICINE, GENERAL & INTERNAL
Bilal Saeed, Samah A Omar, Robert Jones, Christopher J Haas
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引用次数: 0

摘要

多发性骨髓瘤(MM)的特点是单克隆免疫球蛋白的产生导致广泛的骨骼破坏和肾功能障碍。轻链多发性骨髓瘤(LCMM)影响15%的MM患者,总体预后较差。皮肤表现不常见,很少合并I型冷球蛋白血症(CG)。在此,我们报告一例以κ-为主的LCMM合并I型CG的非典型病例,患者为80岁男性,入院前两个月出现累及面部、远端肢体和口咽部的非漂白性坏死性皮疹和溃疡。入院时,检查显示κ-轻链过多,游离κ/λ比升高,冷球蛋白升高,急性肾损伤。骨髓活检显示60%的浆细胞以κ-轻链为主。LCMM的皮肤表现,如肢端发绀和远端坏疽,表明疾病晚期,这些发现应引起对其他合并症病理的怀疑,包括冷球蛋白血症,这可能有助于指导早期开始治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Light Chain Myeloma Precipitating Cryoglobulinemic Vasculitis.

Light Chain Myeloma Precipitating Cryoglobulinemic Vasculitis.

Light Chain Myeloma Precipitating Cryoglobulinemic Vasculitis.

Light Chain Myeloma Precipitating Cryoglobulinemic Vasculitis.

Multiple Myeloma (MM) is characterized by monoclonal immunoglobulin production leading to widespread skeletal destruction and renal dysfunction. Light chain multiple myeloma (LCMM) affects 15% of individuals with MM and has an overall poor prognosis. Cutaneous manifestations are uncommon and it is rarely complicated by Type I Cryoglobulinemia (CG). Here we present an atypical case of κ-predominant LCMM complicated by Type I CG in an 80-year-old man who presented with a progressive non-blanching necrotic rash and ulcers involving his face, distal extremities, and oropharynx of two months duration prior to his admission at our facility. On admission to our facility, workup showed an overabundance of κ-light chains, elevated free κ/λ ratio, cryoglobulins, and an acute kidney injury. Marrow biopsy demonstrated 60% plasma cells with κ-light chain predominance. Cutaneous manifestations such as acral cyanosis and distal gangrene in LCMM indicate late stages of the disease, and such findings should raise suspicion for additional comorbid pathologies, including cryoglobulinemia, which could help direct earlier initiation of treatment.

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来源期刊
自引率
0.00%
发文量
106
审稿时长
17 weeks
期刊介绍: JCHIMP provides: up-to-date information in the field of Internal Medicine to community hospital medical professionals a platform for clinical faculty, residents, and medical students to publish research relevant to community hospital programs. Manuscripts that explore aspects of medicine at community hospitals welcome, including but not limited to: the best practices of community academic programs community hospital-based research opinion and insight from community hospital leadership and faculty the scholarly work of residents and medical students affiliated with community hospitals.
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