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瓦尔登斯特伦巨球蛋白血症中的贫血:一种罕见疾病的罕见表现。

Anaemia in Waldmann's disease: A rare presentation of a rare disease.

作者信息

El-Etreby Shahira A, Altonbary Ahmed Y, Sorogy Mohamed El, Elkashef Wagdi, Mazroa Jehan A, Bahgat Monir H

机构信息

Shahira A El-Etreby, Ahmed Y Altonbary, Mohamed El Sorogy, Wagdi Elkashef, Jehan A Mazroa, Monir H Bahgat, Divisions of Hepatology and Gastroenterology, Pathology, and Diagnostic Radiology, Specialized Medical Hospital and Gastrointestinal Surgery Center, Mansoura 35516, Egypt.

出版信息

World J Gastrointest Endosc. 2015 May 16;7(5):567-72. doi: 10.4253/wjge.v7.i5.567.

Abstract

A 32-year-old female presented with 5-year history of iron deficiency anemia, marked pallor and edema of both lower limbs. Laboratory investigations including complete blood count, blood film, iron studies, lipid profile, ascitic fluid analysis, test of stool for occult blood and alpha 1 anti-trypsin. Upper, lower gastrointestinal (GIT) endoscopies, and enteroscopy were performed. Imaging techniques as abdominal ultrasonography and computed tomography were done. Echocardiography, lymph node biopsy and bone marrow examination were normal. The case was diagnosed as Waldmann's disease with protein losing enteropathy and recurrent GIT bleeding. Management started with low fat diet with medium chain triglyceride, octreotide 200 μg twice a day, tranexamic acid and blood transfusion. Then, exploratory laparotomy with pathological examination of resected segment was done when recurrent GIT bleeding occurred and to excluded malignant transformation.

摘要

一名32岁女性,有5年缺铁性贫血病史,伴有明显面色苍白及双下肢水肿。进行了包括全血细胞计数、血涂片、铁代谢检查、血脂谱、腹水分析、粪便潜血试验及α1抗胰蛋白酶检测在内的实验室检查。还进行了上、下消化道内镜检查及小肠镜检查。采用了腹部超声和计算机断层扫描等影像学技术。超声心动图、淋巴结活检及骨髓检查均正常。该病例被诊断为伴有蛋白丢失性肠病和反复消化道出血的瓦尔德曼病。治疗首先采用低脂饮食并补充中链甘油三酯,奥曲肽200μg每日两次,氨甲环酸及输血治疗。之后,当反复出现消化道出血时,进行了剖腹探查并对切除段进行病理检查,以排除恶性转化。

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本文引用的文献

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Intestinal lymphangiectasia presenting as abdominal mass.表现为腹部肿块的肠淋巴管扩张症。
Gastrointest Endosc. 2007 Mar;65(3):522-3, discussion 523. doi: 10.1016/j.gie.2006.10.026.
8
[Necrolytic migratory erythema in Waldmann's disease].[沃尔德曼病中的坏死性游走性红斑]
Ann Dermatol Venereol. 2006 Aug-Sep;133(8-9 Pt 1):693-6. doi: 10.1016/s0151-9638(06)70994-2.
9
Bilateral breast edema in intestinal lymphangiectasia.肠淋巴管扩张症中的双侧乳腺水肿。
Breast J. 2005 Sep-Oct;11(5):360. doi: 10.1111/j.1075-122X.2005.21578.x.

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