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轻链型淀粉样变性引起的双侧颌下和舌下联合肿胀、巨舌及腕管综合征。

Combined bilateral submandibular and sublingual swelling, macroglossus, and carpal tunnel syndrome caused by light chain amyloidosis.

作者信息

Jønsson V, Rasmussen N, Juhl B R, Gimsing P, Vorstrup S

机构信息

Department of Hematology, University of Copenhagen, Denmark.

出版信息

Ear Nose Throat J. 1998 Feb;77(2):95-8, 100-1.

PMID:9509722
Abstract

Three cases of light chain kappa amyloidosis in multiple myeloma patients are described with remarkable involvement of the tongue and swelling of the sublingual and submandibular regions, and without signs of nephropathy despite Bence Jones kappa proteinuria. All three patients had carpal tunnel syndrome at the beginning of their disease course and only moderate gastrointestinal involvement. Primarily for prognostic reasons, amyloidosis should be suspected in such cases, even in the presence of these highly unusual manifestations, and the diagnosis should be confirmed by unambigously-positive biopsies.

摘要

本文描述了3例多发性骨髓瘤患者发生的轻链κ型淀粉样变性,其舌部受累显著,舌下和颌下区域肿胀,尽管存在本-周蛋白κ型蛋白尿,但无肾病迹象。所有3例患者在病程开始时均有腕管综合征,仅伴有中度胃肠道受累。主要出于预后考虑,即使存在这些非常不寻常的表现,在此类病例中也应怀疑淀粉样变性,诊断应通过明确阳性的活检来证实。

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