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[刚果红阴性轻链病伴顽固性腹泻]

[Congo red-negative light chain disease with intractable diarrhea].

作者信息

Raml A, Bogner S, Hubmann R, Grafinger P, Biesenbach G

机构信息

2. Medizinische Abteilung, Allgemeines Lehrkrankenhaus Linz, Osterreich.

出版信息

Internist (Berl). 2005 Apr;46(4):447-51. doi: 10.1007/s00108-004-1344-y.

DOI:10.1007/s00108-004-1344-y
PMID:15696285
Abstract

A 56-year-old man was admitted due to chronic diarrhea with progressive weight loss (30 kg within 1 year). All results of medical investigations were normal. The suspected diagnosis of a neuroendocrinological neoplasm could not be established; there was also no evidence for a lymphoma or amyloidosis. Chronic diarrhea and weight loss persisted over the ensuing weeks. Additionally, impairment of renal function and heart insufficiency with consecutive pericardial effusion as well as peripheral facial paralysis and peripheral neuropathy could be observed. Six months after hospital admission, the patient died due to progressive multiple organ failure. Postmortem examination revealed normal bone marrow. Only with additional immunohistochemical investigations of all organs could the diagnosis of a systemic Congo red-negative light chain disease be established.

摘要

一名56岁男性因慢性腹泻伴体重进行性减轻(1年内减轻30千克)入院。所有医学检查结果均正常。无法确诊为神经内分泌肿瘤;也没有淋巴瘤或淀粉样变性的证据。在接下来的几周里,慢性腹泻和体重减轻持续存在。此外,还观察到肾功能损害、心力衰竭伴心包积液,以及周围性面瘫和周围神经病变。入院6个月后,患者因进行性多器官衰竭死亡。尸检显示骨髓正常。只有对所有器官进行额外的免疫组织化学检查,才能确诊为系统性刚果红阴性轻链病。

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1
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