Primary Lymphangiectasia
Joel K. Greenson, MD
Key Facts
Clinical Issues
Presentation is often within 1st 3 years of life, although some patients present later
Decreased serum albumin, protein, and gammaglobulins
Poor B-cell function, decreased antibody responses
Protein loss leads to hypoproteinemia and lymphopenia, which leads to infectious complications
Microscopic Pathology
Dilated lacteals within mucosa
Top Differential Diagnoses
Secondary lymphangiectasia
Due to constrictive pericarditis, local obstruction (tumor), radiation, cardiac surgery
Identical to PL on biopsy; only distinguished by history
 Gross photograph shows greatly dilated lymphatic channels  on the serosa of the small intestine. (Courtesy John H. Yardley, MD.) |
 Hematoxylin & eosin shows the tip of a villus with dilated lacteals  . |
TERMINOLOGY
Abbreviations
Primary lymphangiectasia (PL)
Synonyms
Waldmann disease
Primary intestinal lymphangiectasia
Definitions
Dilated lacteals and lymphatic channels in small bowel leading to protein-losing enteropathy
ETIOLOGY/PATHOGENESIS
Developmental Anomaly
Some cases are congenital &/or familial; others seem to be acquired
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