The therapeutic response in Gorham’s syndrome to the beta-blocking agent propranolol is correlated to VEGF-A, but not to VEGF-C or FLT1 expression - Inserm - Institut national de la santé et de la recherche médicale Access content directly
Journal Articles BMC Research Notes Year : 2015

The therapeutic response in Gorham’s syndrome to the beta-blocking agent propranolol is correlated to VEGF-A, but not to VEGF-C or FLT1 expression

Abstract

BACKGROUND: Gorham's syndrome is a rare illness of unknown etiology. It is characterized by a local proliferation of blood or lymphatic vessels that in bones leads to progressive resorption and destruction. The cause of the disease is not elucidated, and therapeutic options remain limited. CASE PRESENTATION: We report herein the case of a young female Caucasian patient aged 18 years with diffuse Gorham syndrome. In tissue specimens angiogenesis and massive lymphangiogenesis as well as the expression of vascular endothelial growth factor-A (VEGF-A) and neuropilins was observed. Lymphangiogenesis is a prominent feature of the disease and a number of lymphatic markers were found to be expressed, however only VEGF-A, but not vascular endothelial growth factor-C (VEGF-C) was found to be elevated in the circulation. Circulating levels of soluble VEGF receptor-1 were also not elevated. Furthermore, the patient responded favorably and the disease was stabilized following treatment with the beta-blocking agent Propranolol alone which acts on VEGF-A alone, but not on soluble VEGF receptor-1 levels. CONCLUSION: This suggests that the disease is dependent on VEGF-A, but on neither VEGF-C, the major driver of lymphangiogenesis, nor FLT1. Furthermore, Propranolol acts on VEGF-A but not FLT1 expression.

Domains

Hematology
Fichier principal
Vignette du fichier
13104_2015_Article_1259.pdf (1.17 Mo) Télécharger le fichier
Origin : Publication funded by an institution
Loading...

Dates and versions

inserm-02076558 , version 1 (22-03-2019)

Identifiers

Cite

Jessica Baud, Abderrahim Lomri, Denis Graber, Andreas Bikfalvi. The therapeutic response in Gorham’s syndrome to the beta-blocking agent propranolol is correlated to VEGF-A, but not to VEGF-C or FLT1 expression. BMC Research Notes, 2015, 8, pp.333. ⟨10.1186/s13104-015-1259-9⟩. ⟨inserm-02076558⟩

Collections

INSERM
50 View
47 Download

Altmetric

Share

Gmail Facebook X LinkedIn More