Incomplete ileus and hemafecia as the presenting features of multi-organ involved primary systemic AL amyloidosis: a rare case report

AL Amyloidosis is known to be a systemic disease affecting multiple organs and tissue while it's rare that patients present with gastrointestinal symptoms at first and later develop multiple-organ dysfuction. Clinical signs are not specific and the diagnosis is rarely given before performing im...

Full description

Saved in:
Bibliographic Details
Published in:BMC gastroenterology 2017-06, Vol.17 (1), p.72-72, Article 72
Main Authors: Tian, Li, Tang, Anliu, Zhang, Xian, Mei, Zhen, Liu, Fen, Li, Jingbo, Li, Xiayu, Ai, Feiyan, Wang, Xiaoyan, Shen, Shourong
Format: Article
Language:English
Subjects:
Abdomen
Abdominal Pain - etiology
AL amyloidosis
Amyloidogenesis
Amyloidosis
Amyloidosis - complications
Amyloidosis - diagnosis
Amyloidosis - drug therapy
Bone marrow biopsy
Care and treatment
Case Report
Case reports
Case studies
Chemotherapy
Complications and side effects
Congo red stain
Dexamethasone
Diagnosis
Disease
Endoscopy
Gastroenterology
Gastrointestinal Hemorrhage - etiology
Health aspects
Histopathology
Humans
Ileal Diseases - etiology
Ileus - etiology
Immunofixation
Laboratories
Light
Male
Melphalan
Microscopy
Middle Aged
Organs
Pain
Patients
Peptides
Potassium
Proteins
Recurrence
Skin
Thalidomide
Typing
Ulcers
Urine
Vomiting - etiology
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:AL Amyloidosis is known to be a systemic disease affecting multiple organs and tissue while it's rare that patients present with gastrointestinal symptoms at first and later develop multiple-organ dysfuction. Clinical signs are not specific and the diagnosis is rarely given before performing immunofixation and endoscopy with multiple biopsies. We would like to emphasize the value of precise diagnostic process of AL amyloidosis. In this case report, we describe a 56-year-old man who presented with recurrent periumbilical pain for 4 months and gradually worsened over a month. After a series of tests, he was finally diagnosed with primary systemic AL amyloidosis. He was treated with a chemotherapy regimen (Melphalan, dexamethasone and thalidomide) achieving a good clinical response. On account of the high misdiagnosis rate, establishing the most precise diagnosis in first time with typing amyloidogenic protein becomes increasingly vital. Although the presenting feature is usually nonspecific, AL amyloidosis ought to be considered when multiple organs are involved in a short period.
ISSN:1471-230X
1471-230X
DOI:10.1186/s12876-017-0628-3