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一种伴有多神经病变、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变的综合征(POEMS综合征)

An Unfortunate Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Change (POEMS).

作者信息

Afridi Faraz, Otoya Jorge, Bunting Samantha F, Chaaya Gerard

机构信息

Internal Medicine, University of Central Florida College of Medicine.

Hematology and Medical Oncology, Osceola Regional Medical Center.

出版信息

Cureus. 2017 Mar 8;9(3):e1086. doi: 10.7759/cureus.1086.

DOI:10.7759/cureus.1086
PMID:28405536
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5384847/
Abstract

POEMS syndrome is an acronym for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes, which is a rare paraneoplastic disease of monoclonal plasma cells. A mandatory criterion to diagnose POEMS syndrome is the presence of a monoclonal plasma cell dyscrasia in which plasma cell leukemia is the most aggressive form. Early identification of the features of the POEMS syndrome is critical for patients to identify an underlying plasma cell dyscrasias and to reduce the morbidity and mortality of the disease by providing early therapy. We present a case of a 64-year-old male who presented with non-specific symptoms and was found to have primary plasma cell leukemia, which was part of his unfortunate POEMS syndrome.

摘要

POEMS综合征是多发性神经病、脏器肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变的首字母缩写,是一种罕见的单克隆浆细胞副肿瘤性疾病。诊断POEMS综合征的一个必要标准是存在单克隆浆细胞异常增生,其中浆细胞白血病是最具侵袭性的形式。早期识别POEMS综合征的特征对于患者识别潜在的浆细胞异常增生并通过早期治疗降低疾病的发病率和死亡率至关重要。我们报告一例64岁男性患者,他出现非特异性症状,被发现患有原发性浆细胞白血病,这是其不幸的POEMS综合征的一部分。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea21/5384847/a5ef88a2878b/cureus-0009-00000001086-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea21/5384847/11eba13d5662/cureus-0009-00000001086-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea21/5384847/74b2fc1406c5/cureus-0009-00000001086-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea21/5384847/98f1faece12e/cureus-0009-00000001086-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea21/5384847/1d589a7e87eb/cureus-0009-00000001086-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea21/5384847/a5ef88a2878b/cureus-0009-00000001086-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea21/5384847/11eba13d5662/cureus-0009-00000001086-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea21/5384847/74b2fc1406c5/cureus-0009-00000001086-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea21/5384847/98f1faece12e/cureus-0009-00000001086-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea21/5384847/1d589a7e87eb/cureus-0009-00000001086-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea21/5384847/a5ef88a2878b/cureus-0009-00000001086-i05.jpg

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