open access

Vol 26, No 4 (2020)
Case report
Published online: 2021-03-22
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Primary intestinal lymphangiectasia with lymphedema of lower extremities

Kamil Klimas, Katarzyna Drożdż, Angelika Chachaj, Andrzej Szuba
DOI: 10.5603/AA.2020.0021
·
Acta Angiologica 2020;26(4):140-143.

open access

Vol 26, No 4 (2020)
Case reports
Published online: 2021-03-22

Abstract

Primary intestinal lymphangiectasia (PIL), also known as Waldmann’s disease, is a rare disorder manifested
by the presence of dilated intestinal lymphatic ducts and leading to protein-losing enteropathy. PIL usually
presents early in childhood; however, rarely may be also diagnosed in adults. Suggestive laboratory findings
include hypoproteinemia, hypoalbuminemia, and hypogammaglobulinemia. Peripheral pitting edema due to
hypoalbuminemia is the main clinical feature. Peripheral lymphedema is a less common symptom. We present
a case of a 23-year-old woman with lymphedema of lower extremities and PIL diagnosed in childhood.

Abstract

Primary intestinal lymphangiectasia (PIL), also known as Waldmann’s disease, is a rare disorder manifested
by the presence of dilated intestinal lymphatic ducts and leading to protein-losing enteropathy. PIL usually
presents early in childhood; however, rarely may be also diagnosed in adults. Suggestive laboratory findings
include hypoproteinemia, hypoalbuminemia, and hypogammaglobulinemia. Peripheral pitting edema due to
hypoalbuminemia is the main clinical feature. Peripheral lymphedema is a less common symptom. We present
a case of a 23-year-old woman with lymphedema of lower extremities and PIL diagnosed in childhood.

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Keywords

lymphatics, lymphedema, protein-losing entheropathy, hypoalbuminemia

About this article
Title

Primary intestinal lymphangiectasia with lymphedema of lower extremities

Journal

Acta Angiologica

Issue

Vol 26, No 4 (2020)

Article type

Case report

Pages

140-143

Published online

2021-03-22

DOI

10.5603/AA.2020.0021

Bibliographic record

Acta Angiologica 2020;26(4):140-143.

Keywords

lymphatics
lymphedema
protein-losing entheropathy
hypoalbuminemia

Authors

Kamil Klimas
Katarzyna Drożdż
Angelika Chachaj
Andrzej Szuba

References (19)
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