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皮肤血管炎:双克隆性淋巴结浆细胞发育异常的一种不寻常表现。

Cutaneous Vasculitis: An Unusual Presentation of a Biclonal Nodal Plasma Cell Dyscrasia.

作者信息

Swan D, Murphy M, Elhassadi E

机构信息

Haematology Department, University Hospital Waterford, Regional Cancer Center South East, University College Cork, Cork, Ireland.

Pathology Department, University Hospital Waterford, Regional Cancer Center South East, University College Cork, Cork, Ireland.

出版信息

Case Rep Hematol. 2017;2017:8152610. doi: 10.1155/2017/8152610. Epub 2017 Sep 13.

DOI:10.1155/2017/8152610
PMID:29057130
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5615981/
Abstract

We describe an unusual case of a biclonal nodal plasma cell dyscrasia, presenting with a vasculitic rash, end-organ damage, and cytopenias. Serum protein electrophoresis demonstrated a biclonal kappa-restricted paraprotein, with a negative skeletal survey and no bone marrow disease. Fluorodeoxyglucose-PET-CT (FDG-PET-CT) revealed nodal involvement, which was not appreciable clinically, and facilitated biopsy, confirming the diagnosis of a nodal plasmacytoma. Complete biochemical response and resolution of the vasculitic rash were achieved with bortezomib-based therapy.

摘要

我们描述了一例罕见的双克隆性淋巴结浆细胞发育异常病例,表现为血管炎性皮疹、终末器官损害和血细胞减少。血清蛋白电泳显示双克隆性κ轻链限制的副蛋白,骨骼检查阴性且无骨髓疾病。氟脱氧葡萄糖-PET-CT(FDG-PET-CT)显示有淋巴结受累,临床上未发现,有助于活检,确诊为淋巴结浆细胞瘤。基于硼替佐米的治疗实现了完全生化缓解和血管炎性皮疹消退。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee5a/5615981/555c314e4d75/CRIHEM2017-8152610.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee5a/5615981/bd77f0c9a37a/CRIHEM2017-8152610.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee5a/5615981/555c314e4d75/CRIHEM2017-8152610.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee5a/5615981/bd77f0c9a37a/CRIHEM2017-8152610.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee5a/5615981/555c314e4d75/CRIHEM2017-8152610.002.jpg

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本文引用的文献

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Role of F-FDG PET/CT in the diagnosis and management of multiple myeloma and other plasma cell disorders: a consensus statement by the International Myeloma Working Group.F-FDG PET/CT 在多发性骨髓瘤和其他浆细胞疾病的诊断和管理中的作用:国际骨髓瘤工作组的共识声明。
Lancet Oncol. 2017 Apr;18(4):e206-e217. doi: 10.1016/S1470-2045(17)30189-4.
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Clinical characteristics and outcomes in biclonal gammopathies.双克隆丙种球蛋白病的临床特征与转归
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Image interpretation criteria for FDG PET/CT in multiple myeloma: a new proposal from an Italian expert panel. IMPeTUs (Italian Myeloma criteria for PET USe).
多发性骨髓瘤中FDG PET/CT的图像解读标准:来自意大利专家小组的新提议。IMPeTUs(意大利PET使用的骨髓瘤标准)
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Br J Haematol. 2014 Oct;167(1):131-3. doi: 10.1111/bjh.12926. Epub 2014 May 7.
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Biclonal IgD and IgM Plasma Cell Myeloma: A Report of Two Cases and a Literature Review.双克隆IgD和IgM浆细胞骨髓瘤:两例报告及文献综述
Case Rep Hematol. 2013;2013:293150. doi: 10.1155/2013/293150. Epub 2013 Nov 18.
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The spectrum of paraneoplastic cutaneous vasculitis in a defined population: incidence and clinical features.特定人群中副肿瘤性皮肤血管炎的谱系:发病率及临床特征
Medicine (Baltimore). 2013 Nov;92(6):331-343. doi: 10.1097/MD.0000000000000009.
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FDG PET or PET/CT for detecting intramedullary and extramedullary lesions in multiple Myeloma: a systematic review and meta-analysis.18F-FDG PET 或 PET/CT 检测多发性骨髓瘤髓内和髓外病变:系统评价和荟萃分析。
Clin Nucl Med. 2012 Sep;37(9):833-7. doi: 10.1097/RLU.0b013e31825b2071.
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Haematologica. 2012 Nov;97(11):1761-7. doi: 10.3324/haematol.2012.065698. Epub 2012 Jun 11.
9
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Anticancer Res. 2010 Sep;30(9):3791-4.
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