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Start free with EleplanPrimary intestinal lymphangiectasia
ORPHA:90362Disease
Also called Waldmann disease
What it is
A rare intestinal disease characterized by dilated intestinal lacteals which cause lymph leakage into the small bowel lumen. Clinical manifestations include edema related to hypoalbuminemia (protein-losing gastro-enteropathy), asthenia, moderate diarrhea, lymphedema, serous effusion and failure to thrive in children.
Key facts
- Age of onset
- All ages
- Classified as
- Disease
Signs and symptoms
Very common80–99%
6Common30–79%
9Sometimes5–29%
17- Abdominal pain
- Ascites
- Decreased circulating IgA level
- Decreased circulating IgG level
- Decreased circulating total IgM
- Decreased proportion of CD3-positive T cells
- Generalized edema
- Hypocalcemia
and 9 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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