Vascular Endothelial Growth Factor Serum Levels Are Elevated in Patients with Hereditary Hemorrhagic Telangiectasia

Abstract

Background: Hereditary hemorrhagic telangiectasia (HHT) is a genetic angiodysplasia affecting multiple organs. Two genes involved in the transduction of TGF-β signalling are responsible for HHT. An additional role for vascular endothelial growth factor (VEGF) has been proposed. Serum VEGF, which has been evaluated in several diseases characterized by aberrant angiogenesis, has never been measured in patients with HHT. Aims: To evaluate VEGF serum levels in HHT patients as compared to normal subjects. Materials and Methods: 32 HHT patients (age 47.7 ± 16.7 years) and a control group of 37 healthy subjects (age 48.2 ± 15.5 years) were entered in the study. Each patient underwent serum VEGF dosage using a commercial ELISA specific for the human molecule. Results: The serum level of VEGF in HHT patients was 196.3 ± 103.2 pg/ml, while it was 152.0 ± 84.1 pg/ml in the control group. Statistical analysis showed that serum VEGF was significantly higher in HHT patients than in the controls (p < 0.031). Conclusions: According to a study performed in a murine model, persistence of the activation phase of angiogenesis might be responsible for an increased production of several angiogenic factors, in particular VEGF, in HHT. Our work is the first to suggest an increased expression of VEGF in the serum of subjects with HHT in agreement with the stimulation of VEGF synthesis proposed in the murine model.

A retrospective analysis of low dose, intranasal injected bevacizumab (Avastin) in hereditary haemorrhagic telangiectasia

“…The reason for this is thought to be a defect in the endoglin or ALK1 protein [12][13][14]. VEGF is particularly interesting, in this respect, because there is already an anti-VEGF monoclonal antibody (bevacizumab) on the market which is used to treat metastatic malignancies, e.g.…”

Successful therapy with bevacizumab in a case of hereditary hemorrhagic telangiectasia

“…HTT is often associated with increased levels of VEGF. 5,6 A case of a female patient with HHT and chronic GI bleeding is presented. The patient required iron supplementation and multiple blood transfusions.…”

Vascular Endothelial Growth Factor Plasma Levels Are Significantly Elevated in Patients with Cerebral Arteriovenous Malformations

“…VEGF plasma levels were shown to be correlated with different stages and prognosis of malignant tumours or haematological diseases [15, 16,23,24,25,26]. Furthermore it was investigated in patients with vascular disorders such as hereditary haemorrhagic telangiectasia (HHT) also known as Osler-Rendu-Weber syndrome [9, 10, 27, 28]. Elevated VEGF plasma levels and enhanced VEGF expression in HHT tissue samples suggested that the VEGF seems to play a major role in the pathogenesis of HHT [27].…”