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Case Reports
. 2018 Apr 17;6(6):1098-1100.
doi: 10.1002/ccr3.1536. eCollection 2018 Jun.

A young woman with steroid-responsive, IgG4-positive plasma cell-enriched cystic lymphangioma and chylous ascites

Alaa A Al Zaki  1 , Shawna L Mann  2 , Mollie N Carruthers  3 , Graham W Slack  4 , Luke Y C Chen  1
Affiliations
Case Reports

A young woman with steroid-responsive, IgG4-positive plasma cell-enriched cystic lymphangioma and chylous ascites

Alaa A Al Zaki et al. Clin Case Rep. .

Abstract

Lymphangiomas are benign tumors of the lymphatic vessels, which can be inflammatory and occasionally steroid-responsive. IgG4-related disease (IgG4-RD) is a recently defined fibro-inflammatory condition. We describe a novel association between reactive IgG4+ plasma cells and cystic lymphangioma in a young woman who had a dramatic clinical response to steroids.

Keywords: IgG4‐related disease; inflammation; lymphangioma; steroid.

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Figures

Figure 1
Figure 1
The pelvic mass is composed of necrotic tissue surrounded by a thin rim of fibrous tissue and chronic inflammation (hematoxylin–eosin, original magnification 20x).
Figure 2
Figure 2
An immunohistochemical stain shows the vast majority (>95%) of plasma cells are positive for IgG4 (original magnification 200x).
Figure 3
Figure 3
The chronic inflammatory infiltrate is made up of abundant small mature plasma cells (>100 per high power field) with a few admixed small mature lymphocytes and histiocytes (hematoxylin–eosin, original magnification 200x).
See this image and copyright information in PMC

References

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