Tolerability, safety and effectivity of Bevacizumab (Avastin) in hereditary hemorrhagic teleangiectasia (HHT) – from the standard therapeutic regime to a long-term therapy

Introduction:

HHT is characterized by an impaired angiogenesis being accompanied by increased levels of VEGF. Systemic therapy with Bevacizumab has been shown to reduce epistaxis, gastrointestinal and cardiac complications. Statements on optimal therapy duration and dosage for an effective treatment with a minimum of adverse effects do not yet exist. In the following we present the application of Bevacizumab as temporary as well as maintenance therapy.

Methods:

4 patients received 5 mg/kg body weight Bevacizumab every 2 weeks in 6 cycles. 2 patients subsequently received a maintenance therapy with 2.5 mg/kg body weight Bevacizumab every 1 – 3 months. We evaluated frequency and duration of epistaxis as well as hemoglobine (Hb) and ferritin (Fe) levels before and after therapy.

Results:

In patient 1 the intensity and quantity of epistaxis as well as the neccessity for erythrocyte concentrates could be reduced. Hb and Fe levels improved. Patient 2 exhibited a moderate reduction of duration and intensity of epistaxis. Again, Hb and Fe levels increased. During maintenance therapy, conducted with good tolerability, Hb levels could be stabilized in cases 3 and 4. There was no further need for erythrocyte concentrates. Maintenance therapy could be continued for 36 months without relevant sideeffects.

Conclusions:

The symptom-related effectivity of Bevacizumab is rather variable. With the regimen applied no significant adverse effects were observed. Also maintenance therapy proved to be safe. Further studies on identifying patient-related predictive factors as to modify the therapeutic regimen are recommended.

Publication History

Publication Date:
23 April 2019 (online)