Emerging role of bevacizumab in management of patients with symptomatic hepatic involvement in Hereditary Hemorrhagic Telangiectasia

Chavan, Ajay; Schumann-Binarsch, Silke; Schmuck, Bernhard; Oltmer, Franziska; Geisthoff, Urban W.; Hoppe, Florian; Wirsching, Kornelia; Klempnauer, J.; Manns, Michael P.; Thomas, Rohit Philip; Köhne, Claus‐Henning

doi:10.1002/ajh.24878

Cited by 19 publications

(8 citation statements)

References 6 publications

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“…These measures can be performed in-office by local anesthesia, but excessive bleeding can require interventions (laser therapy, coagulation and the closure of feeders, e.g., closure of the sphenopalatine artery, or sclerotherapy) under general anesthesia [ 16 , 20 , 22 ]. If those measures cannot improve the severity of epistaxis or if the involvement of other organs requires treatment, such as the gastrointestinal tract or the liver, drug therapies (tranexamic acid, bevacizumab, thalidomide and tacrolimus) are available [ 10 , 15 , 23 , 24 , 25 ]. The Osler Calendar documents the therapeutic measures since the last patient contact, including iron supplements, blood transfusions, nasal packing, laser therapy, coagulation, surgical interventions, and drug treatments ( Figure 3 A, right).…”

Section: Osler Calendar Content and Resultsmentioning

confidence: 99%

Hereditary Hemorrhagic Telangiectasia: Success of the Osler Calendar for Documentation of Treatment and Course of Disease

Seebauer¹,

Freigang²,

Schwan³

et al. 2021

JCM

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Hereditary hemorrhagic telangiectasia (HHT; Rendu-Osler-Weber syndrome) affects the capillary and larger vessels, leading to arteriovenous shunts. Epistaxis is the main symptom impairing quality of life. The aim of the Osler Calendar is to offer information about the extent of the systemic disease and the current state of treatment. A care plan with information on the rare disease and self-treatment of epistaxis was created. Organ examinations and ongoing treatments were recorded. A questionnaire documents the treatment success, including patient satisfaction, frequency of hemorrhage and hemoglobin levels. The patients using the Osler Calendar for at least one year (n = 54) were surveyed. Eighty-five percent of patients (n = 46) used the calendar to gain information about HHT. Seventy-two percent (n = 39) used the Osler Calendar for instructions on the self-treatment of nosebleeds. The calendar increased patients’ understanding for the need for organ screenings from 48% (n = 26) to 81% (n = 44). Seventy-nine percent (n = 43) of patients confirmed that the Osler Calendar documented their therapeutic process either well or very well. Fifty-two percent (n = 28) saw an improvement in the therapeutic process due to the documentation. The Osler Calendar records the individual intensity of the disease and facilitates the communication between attending physicians. It is a tool for specialists to review treatment strategies. Furthermore, the calendar enhances patients’ comprehension of their condition.

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Section: Osler Calendar Content and Resultsmentioning

confidence: 99%

Hereditary Hemorrhagic Telangiectasia: Success of the Osler Calendar for Documentation of Treatment and Course of Disease

Seebauer¹,

Freigang²,

Schwan³

et al. 2021

JCM

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“…Bleeding (epistaxis and/or GI bleeding) was the treatment indication in 106 patients (23,(26)(27)(28)(29)(30) , of whom 92 (87 %) had a positive effect of treatment with reduced bleeding, reduced transfusion needs, and/or increased levels of hemoglobin. Fifty-six patients from three studies (17,29,31) were primarily treated with systemic IV bevacizumab due to symptomatic hepatic involvement. Forty-nine of these (88%) also benefited from the treatment with reduced bleeding tendency.…”

Section: Synthesis Of Resultsmentioning

confidence: 99%

“…The repeated search in September 2018 resulted in one extra case report (25) and one extra case series (26) . Characteristics of the eight case series (17,23,(26)(27)(28)(29)(30)(31) and the 33 case reports (25, included in the present study are summarized in Table 3 and Table 4, respectively, including study type, number of patients, gender,…”

Section: Study Selection and Characteristicsmentioning

confidence: 99%

Does severe bleeding in HHT patients respond to intravenous bevacizumab? Review of the literature and case series

Rosenberg

Fialla

Kjeldsen

et al. 2019

Rhin

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Background: Hereditary Haemorrhagic Telangiectasia (HHT) is an autosomal dominant genetic disorder, with a wide variety of clinical manifestations due to the presence of multiple arteriovenous manifestations. Severe bleeding from the gastrointestinal (GI) tract and/or epistaxis presents a significant problem in a subgroup of patients and systemic bevacizumab, an angiogenesis inhibitor, has been suggested to benefit these patients. Objective:To perform a review of the literature concerning the efficacy of systemic bevacizumab in treatment of bleeding from the nose or GI tract in patients with HHT, including patients from our own HHT-center.Methods: A literature review was performed using the guideline "Preferred Reporting Items for systematic Reviews and Meta-Analysis statement" (PRISMA). Results:After careful selection, we finally analysed the results of eight case series and 33 case reports. Among 195 patients 171 (88%) had reduced bleeding after bevacizumab. Conclusions:Based on the literature review and data from our own case series, systemic bevacizumab is very promising as treatment for HHT patients with severe epistaxis and/or GI-bleeding. However, care should be taken using bevacizumab, a potent angiogenesis inhibitor; long-term side effects have not been studied in this population. A randomized controlled study is warranted to support the results in HHT patients.

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“…Because of this, epistaxis grade, a less precise evaluation of severity that was available for all patients during both pretreatment and maintenance, was used instead to allow for uniformity of data and subsequent data analysis. Epistaxis grade has been used in other HHT studies as well . Examination of the epistaxis grade data in the maintenance period for each patient (Table ) reveals that 85% of patients had grade 0 (no epistaxis) or grade 1 (mild symptoms, intervention not indicated).…”

Section: Discussionmentioning

confidence: 99%

Systemic bevacizumab for the treatment of chronic bleeding in hereditary haemorrhagic telangiectasia

et al. 2018

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Emerging role of bevacizumab in management of patients with symptomatic hepatic involvement in Hereditary Hemorrhagic Telangiectasia

Cited by 19 publications

References 6 publications

Hereditary Hemorrhagic Telangiectasia: Success of the Osler Calendar for Documentation of Treatment and Course of Disease

Hereditary Hemorrhagic Telangiectasia: Success of the Osler Calendar for Documentation of Treatment and Course of Disease

Does severe bleeding in HHT patients respond to intravenous bevacizumab? Review of the literature and case series

Systemic bevacizumab for the treatment of chronic bleeding in hereditary haemorrhagic telangiectasia

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