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Pulmonary and pleural lymphatic endothelial cells from pediatric, but not adult, patients with Gorham-Stout disease and generalized lymphatic anomaly, show a high proliferation rate

Mori, Michiko LU ; Dictor, Michael LU ; Brodszki, Nicholas LU ; López-Gutiérrez, Juan Carlos; Beato, María; Erjefält, Jonas S. LU and Eklund, Erik A. LU (2016) In Orphanet Journal of Rare Diseases 11(1).
Abstract

Background: Gorham-Stout disease (OMIM 123880) and generalized lymphatic anomaly are two rare disorders of lymphendothelial growth in which thoracic involvement with chylothorax is a feared complication. Currently it is believed that both disorders are prenatal malformations that progress slowly after birth. Several pharmaceuticals with antiproliferative properties, including interferon-α-2b, rapamycin and propranolol, have however been shown to affect the disease course in some patients. Deeper knowledge of the growth characteristics of these malformations are therefore needed to guide the clinical approach. Methods: Lymphatic vessels in lung and pleural tissue from both children and adult patients with generalized lymphatic anomaly or... (More)

Background: Gorham-Stout disease (OMIM 123880) and generalized lymphatic anomaly are two rare disorders of lymphendothelial growth in which thoracic involvement with chylothorax is a feared complication. Currently it is believed that both disorders are prenatal malformations that progress slowly after birth. Several pharmaceuticals with antiproliferative properties, including interferon-α-2b, rapamycin and propranolol, have however been shown to affect the disease course in some patients. Deeper knowledge of the growth characteristics of these malformations are therefore needed to guide the clinical approach. Methods: Lymphatic vessels in lung and pleural tissue from both children and adult patients with generalized lymphatic anomaly or Gorham-Stout disease were studied using an immunohistochemical approach, targeting lymphendothelial markers (D2-40/Prox-1) and a proliferation marker (Ki-67). Results: We found significant proliferation and growth in these lesions in pediatric patients but not in adults. Furthermore, the data may suggest that the disease process is at least partly reversible. Conclusions: These malformations of the lymphatic system proliferate at a significant rate long after birth, which could suggest that the clinical approach for children should be different from adults.

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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Chylothorax, Generalized lymphatic anomaly, Gorham-Stout disease, Lymphatic malformation, VEGF-C
in
Orphanet Journal of Rare Diseases
volume
11
issue
1
publisher
BioMed Central
external identifiers
  • Scopus:84975060426
ISSN
1750-1172
DOI
10.1186/s13023-016-0449-4
language
English
LU publication?
yes
id
b38b302f-76ed-4a9c-95f2-3358814e31b3
date added to LUP
2016-07-06 15:04:14
date last changed
2016-09-20 03:09:53
@misc{b38b302f-76ed-4a9c-95f2-3358814e31b3,
  abstract     = {<p>Background: Gorham-Stout disease (OMIM 123880) and generalized lymphatic anomaly are two rare disorders of lymphendothelial growth in which thoracic involvement with chylothorax is a feared complication. Currently it is believed that both disorders are prenatal malformations that progress slowly after birth. Several pharmaceuticals with antiproliferative properties, including interferon-α-2b, rapamycin and propranolol, have however been shown to affect the disease course in some patients. Deeper knowledge of the growth characteristics of these malformations are therefore needed to guide the clinical approach. Methods: Lymphatic vessels in lung and pleural tissue from both children and adult patients with generalized lymphatic anomaly or Gorham-Stout disease were studied using an immunohistochemical approach, targeting lymphendothelial markers (D2-40/Prox-1) and a proliferation marker (Ki-67). Results: We found significant proliferation and growth in these lesions in pediatric patients but not in adults. Furthermore, the data may suggest that the disease process is at least partly reversible. Conclusions: These malformations of the lymphatic system proliferate at a significant rate long after birth, which could suggest that the clinical approach for children should be different from adults.</p>},
  author       = {Mori, Michiko and Dictor, Michael and Brodszki, Nicholas and López-Gutiérrez, Juan Carlos and Beato, María and Erjefält, Jonas S. and Eklund, Erik A.},
  issn         = {1750-1172},
  keyword      = {Chylothorax,Generalized lymphatic anomaly,Gorham-Stout disease,Lymphatic malformation,VEGF-C},
  language     = {eng},
  month        = {05},
  number       = {1},
  publisher    = {ARRAY(0x9893908)},
  series       = {Orphanet Journal of Rare Diseases},
  title        = {Pulmonary and pleural lymphatic endothelial cells from pediatric, but not adult, patients with Gorham-Stout disease and generalized lymphatic anomaly, show a high proliferation rate},
  url          = {http://dx.doi.org/10.1186/s13023-016-0449-4},
  volume       = {11},
  year         = {2016},
}