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Diffuse lymphangiomatosis--a fatal case with atypical skeletal features
Eyal Meltzer1, Elinor Goshen, Eduard Fridman
1Department of Internal Medicine C, The Sheba Medical Center, Tel Hashomer, Israel. emeltzer@post.tau.ac.il
The American Journal of the Medical Sciences
|November 18, 2008
Summary
Diffuse lymphangiomatosis, a rare condition causing organ dysfunction, can be challenging to diagnose. A unique Tc99 bone scan finding may aid in earlier identification of this lymphatic disorder.
Area of Science:
- Medicine
- Radiology
- Pathology
Background:
- Diffuse lymphangiomatosis is a rare, idiopathic condition primarily affecting children, marked by non-neoplastic lymphatic vessel proliferation.
- This condition leads to significant organ dysfunction, chylous effusions, and can be fatal.
- Gorham-Stout syndrome shares features like lymphangiomatosis and chylous effusions but includes massive osteolysis ('vanishing bone disease').
Observation:
- A 33-year-old woman presented with a 5-year history of progressive chylous effusions and organomegaly.
- Extensive diagnostic evaluations ruled out numerous conditions.
- Skeletal radiography showed no osteolytic lesions, but a Tc99 bone scan revealed absent osteoblastic activity in certain bones.
Findings:
- Autopsy confirmed diffuse lymphangiomatosis with histologically normal bone.
- The case highlights an unusual imaging pattern not previously associated with diffuse lymphangiomatosis.
Implications:
- This distinct Tc99 bone scan pattern, if consistently observed, could serve as a valuable diagnostic aid.
- Such an aid may significantly reduce diagnostic delays in diffuse lymphangiomatosis.
- Further research is needed to validate this imaging finding in future cases.
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