Bevacizumab dramatically improves severe hereditary hemorrhagic telangiectasia (HHT) associated bleeding

(Elsevier) Patients with hereditary hemorrhagic telangiectasia (HHT) with severe bleeding, who were treated with intravenous bevacizumab, reported a marked reduction in nose bleeds and gastrointestinal bleeding and were able to stop or considerably reduce blood transfusions, resulting in significantly improved quality of life. A new study published in Mayo Clinic Proceedings provides good quality evidence for the excellent safety profile and efficacy of intravenous bevacizumab in the management of these patients.
Source: EurekAlert! - Medicine and Health - Category: International Medicine & Public Health Source Type: news

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Authors: Galaris G, Thalgott JH, Lebrin FPG Abstract Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder characterized by multi-systemic vascular dysplasia affecting 1 in 5000 people worldwide. Individuals with HHT suffer from many complications including nose and gastrointestinal bleeding, anemia, iron deficiency, stroke, abscess, and high-output heart failure. Identification of the causative gene mutations and the generation of animal models have revealed that decreased transforming growth factor-β (TGF-β)/bone morphogenetic protein (BMP) signaling and increased vascular endothelial growth...
Source: Advances in Experimental Medicine and Biology - Category: Research Tags: Adv Exp Med Biol Source Type: research
This study was carried out in accordance with approval of the Melbourne Health and Walter and Eliza Hall Institute of Medical Research's Human Research Ethics Committee (approval number: 2013.081). All subjects gave written informed consent for participation and publication. Results and Discussion TGF-β signaling in NK cells is associated with: phosphorylation in SMAD2 and 3, inhibition of IL-15-induced metabolism/proliferation, simultaneous downregulation of CD44, CD49e, and Eomes, and upregulation of CD16 and CD49a expression (7, 10). SMAD family member 4 (SMAD4) belongs to the SMAD family of transcription...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
For people with a rare bleeding disorder called "hereditary hemorrhagic telangiectasia," or HHT, simple issues such as a nosebleed can be life-threatening. The disease has no cure. But Dr. Vivek Iyer, a Mayo Clinic pulmonary and critical care physician, found that a drug commonly used for cancer can stop the bleeding and restore quality of [...]
Source: News from Mayo Clinic - Category: Databases & Libraries Source Type: news
Pulmonary arteriovenous fistula (PAVF), a vessel malformation connecting the pulmonary circulation to the systemic circulation while bypassing the pulmonary capillaries, can cause paradoxical cerebral infarction. It is often associated with hereditary hemorrhagic telangiectasia (HHT), a genetic disease characterized by multiple dermal, mucosal, and visceral telangiectasia causing recurrent bleeding. Paradoxical cerebral embolism caused by PAVF without HHT is rare. Here, we report a patient with isolated PAVF who experienced an ischemic stroke caused by a paradoxical embolism from deep venous thrombosis; the patient was suc...
Source: Journal of Stroke and Cerebrovascular Diseases - Category: Neurology Authors: Tags: Case Report Source Type: research
Pulmonary arteriovenous malformations are rare cause for ischemic stroke. British Thoracic Society Clinical Statement considered insufficient evidence of safety or clinical benefit to recommend thrombolysis for stroke with pulmonary arteriovenous malformations. For pulmonary arteriovenous malformations with hereditary hemorrhagic telangiectasia, bleeding risk after thrombolysis is high, while for isolate pulmonary arteriovenous malformations, bleeding risk is much lower. We here present 2 cases of ischemic stroke with isolate pulmonary arteriovenous malformations treated with thrombolysis.
Source: Journal of Stroke and Cerebrovascular Diseases - Category: Neurology Authors: Tags: Case Report Source Type: research
Source: Digestive and Liver Disease - Category: Gastroenterology Authors: Tags: Posters Source Type: research
Pulmonary arteriovenous malformations (PAVMs) are rare and commonly caused by an autosomal dominant disorder known as hereditary hemorrhagic telangiectasia (HHT). HHT is an often-missed vascular disorder causing symptoms of epistaxis, mucosal telangiectasia, AVMs, hemoptysis, dyspnea, and iron deficiency anemia. Large AVMs in the lungs, liver, and brain can be life-threatening. Early diagnosis and intervention are imperative to prevent serious complications of massive hemoptysis, stroke, cerebral abscess, and gastrointestinal bleeding.
Source: The Journal for Nurse Practitioners - Category: Nursing Authors: Tags: Featured Article Source Type: research
Conditions:   Hereditary Hemorrhagic Telangiectasia;   Epistaxis Nosebleed;   Anemia Interventions:   Drug: Pazopanib;   Drug: Placebo oral capsule Sponsor:   Cure HHT Not yet recruiting
Source: - Category: Research Source Type: clinical trials
CONCLUSIONS: Our study showed that most patients with HHT using nasal self-packings could stop the bleeding after a while. Nasal self-packing is a user-friendly and secure method leaving patients more self-confident and independent. PMID: 30739126 [PubMed - as supplied by publisher]
Source: Rhinology - Category: ENT & OMF Tags: Rhinology Source Type: research
Abstract Hereditary hemorrhagic telangiectasia (HHT) is a vascular rare disease characterized by nose and gastrointestinal bleeding, skin and mucosa telangiectasias, and arteriovenous malformations in internal organs. HHT shows an autosomal dominant inheritance and a worldwide prevalence of approximately 1:5000 individuals. In>80% of patients, HHT is caused by mutations in either ENG (HHT1) or ACVRL1 (HHT2) genes, which code for the membrane proteins Endoglin and Activin A Receptor Type II-Like Kinase 1 (ALK1), respectively, both belonging to the TGF-β/BMP signaling pathway. In this work, we describe a nov...
Source: Gene - Category: Genetics & Stem Cells Authors: Tags: Gene Source Type: research
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