Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2021 Jan 18;14(1):e237331.
doi: 10.1136/bcr-2020-237331.

Systemic generalised lymphangiomatosis: unknown aetiology and a challenge to treat

Tess Van Meerhaeghe  1 , Frederik Vandenbroucke  2 , Brigitte Velkeniers  3
Affiliations
Case Reports

Systemic generalised lymphangiomatosis: unknown aetiology and a challenge to treat

Tess Van Meerhaeghe et al. BMJ Case Rep. .

Abstract

We describe a case of a woman diagnosed at the age of 35 years with a generalised mediastinal and abdominal lymphangiomatosis associated with a protein losing enteropathy, who successfully improved when treatment with sirolimus was initiated.

Keywords: cardiovascular system; malabsorption.

PubMed Disclaimer

Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Coronal view of the contrast enhanced CT scans with the selected content of the peritoneal and retroperitoneal cysts, marked in blue. A, B illustrates the lymphangiomatosis at the start (A) and 24 months after start of sirolimus therapy (B). (C, D): Three-D image of the calculated volumes of the selected content as shown in (A, B). This was done for both scans with an interval of 24 months. (C) Before start of therapy—(D) 24 months after start of sirolimus therapy).
See this image and copyright information in PMC

References

    1. Blei F. Lymphangiomatosis: clinical overview. Lymphat Res Biol 2011;9:185–90. 10.1089/lrb.2011.0020 - DOI - PubMed
    1. Rockson SG. Laboratory models for the investigation of lymphangiomatosis. Microvasc Res 2014;96:64–7. 10.1016/j.mvr.2014.08.007 - DOI - PubMed
    1. Wiegand S, Eivazi B, Barth PJ, et al. . Pathogenesis of lymphangiomas. Virchows Arch 2008;453:1–8. 10.1007/s00428-008-0611-z - DOI - PubMed
    1. Ji R-C, Eshita Y, Xing L, et al. . Multiple expressions of lymphatic markers and morphological evolution of newly formed lymphatics in lymphangioma and lymph node lymphangiogenesis. Microvasc Res 2010;80:195–201. 10.1016/j.mvr.2010.04.002 - DOI - PubMed
    1. Kadakia KC, Patel SM, Yi ES, et al. . Diffuse pulmonary lymphangiomatosis. Can Respir J 2013;20:52–4. 10.1155/2013/971350 - DOI - PMC - PubMed

Publication types

LinkOut - more resources