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IgG-κ轻链浆细胞瘤中的胃肠道淀粉样变性。尽管有晚期改变,但仍存在诊断问题

[Gastrointestinal amyloidosis in IgG-chi-light chain plasmacytoma. Diagnostic problems despite advanced changes].

作者信息

Reithmeier A, Trenkwalder P, Kaess H, Lydtin H

机构信息

Medizinische Klinik, Kreiskrankenhauses Starnberg.

出版信息

Dtsch Med Wochenschr. 1995 May 5;120(18):641-5. doi: 10.1055/s-2008-1055390.

DOI:10.1055/s-2008-1055390
PMID:7750431
Abstract

A 50-year-old man with an IgG-chi light chain multiple myeloma stage IIIA, developed--in a phase of low disease activity, after 18 months of an uncomplicated course--marked malabsorption syndrome with 20 kg weight loss, diarrhoea and meteorism. Although the H2-breath test indicated intestinal bacterial colonisation, neither antibacterial treatment with trimethoprim/sulphamethoxazole and metronidazole nor prokinetic treatment with cisapride (30 mg daily) and erythromycin (1 g twice daily) improved the symptoms. Suspected amyloidosis was not demonstrable at first, despite repeated step biopsies of stomach, duodenum and rectum. Amyloidosis of the entire gastrointestinal tract was proven only by repeated biopsies deep into the submucosa. Despite treatment of the underlying disease with melphalan and prednisone (Alexanian's scheme) the amyloidosis advanced further to involve liver, spleen, lung, kidneys and heart. The patient died, 2 years after diagnosis of the multiple myeloma, from recurrent pulmonary emboli due to atrial fibrillation.

摘要

一名50岁男性,患有IgG-κ轻链多发性骨髓瘤IIIA期,在病情平稳的18个月后进入疾病低活动期,出现明显的吸收不良综合征,体重减轻20千克,伴有腹泻和气胀。尽管氢呼气试验显示肠道细菌定植,但使用甲氧苄啶/磺胺甲恶唑和甲硝唑进行抗菌治疗,以及使用西沙必利(每日30毫克)和红霉素(每日2次,每次1克)进行促动力治疗,均未能改善症状。尽管对胃、十二指肠和直肠进行了多次阶梯式活检,但最初并未证实存在疑似淀粉样变性。仅通过深入黏膜下层的反复活检才证实整个胃肠道存在淀粉样变性。尽管按照马法兰和泼尼松(阿列克萨尼安方案)治疗基础疾病,但淀粉样变性仍进一步发展,累及肝脏、脾脏、肺、肾脏和心脏。该患者在诊断为多发性骨髓瘤2年后,因心房颤动导致复发性肺栓塞死亡。

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1
[Gastrointestinal amyloidosis in IgG-chi-light chain plasmacytoma. Diagnostic problems despite advanced changes].IgG-κ轻链浆细胞瘤中的胃肠道淀粉样变性。尽管有晚期改变,但仍存在诊断问题
Dtsch Med Wochenschr. 1995 May 5;120(18):641-5. doi: 10.1055/s-2008-1055390.
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引用本文的文献

1
Multiple myeloma presenting with malabsorption.
Dig Dis Sci. 2007 Aug;52(8):1851-4. doi: 10.1007/s10620-006-9157-6. Epub 2007 Apr 6.