Blood Res.  2014 Sep;49(3):192-195. 10.5045/br.2014.49.3.192.

Hereditary hemorrhagic telangiectasia treated with low dose intravenous bevacizumab

• Wee JW
• Jeon YW
• Eun JY
• Kim HJ
• Bae SB
• Lee KT

• KMID: 2172792
• DOI: http://doi.org/10.5045/br.2014.49.3.192

Abstract

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder that leads to mucocutaneous telangiectasias, epistaxis, and gastrointestinal bleeding. Depending on the severity and manifestation of the disease, various therapeutic modalities have been used, from local bleeding control to surgery or concomitant drug therapy. Several articles under review have presented guidelines for treatment of HHT with bevacizumab as a direct anti-angiogenesis strategy. Still, neither the exact optimal dose nor the minimum effective dose of intravenous bevacizumab in patients with severe HHT has been reported. A 55-year-old man presented with long-standing epistaxis, recent melena, dizziness, and a three-generation family history of chronic epistaxis, anemia, and regular blood transfusions. Treatment with argon plasma coagulation (APC) for the gastrointestinal bleeding failed to raise hemoglobin levels, we considered using the bevacizumab. We report a patient with severe HHT, who was treated with low-dose bevacizumab (2 mg/kg) and improved substantially.

Keyword

Bevacizumab; Hereditary hemorrhagic telangiectasia; Low dose

MeSH Terms

Anemia
Argon Plasma Coagulation
Blood Transfusion
Dizziness
Drug Therapy
Epistaxis
Hemorrhage
Humans
Melena
Middle Aged
Telangiectasia, Hereditary Hemorrhagic*
Telangiectasis
Bevacizumab

Figure

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  Fig. 1 Pedigree of the patient's family. Red box indicates those affected by hereditary hemorrhagic telangiectasia.

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  Fig. 2 (A) The lips and tongue showed mucocutaneous telangiectasias. (B) Numerous telangiectasia lesions of the total gastric mucosa with old blood clots and easy touch bleeding patterns were observed by endoscopic gastroduodenoscopy (EGD). Argon plasma coagulation (APC) provided successful management. (C) Follow-up EGD showed improved telangiectasia of the gastric mucosa after bevacizumab therapy.

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  Fig. 3 Hemoglobin and serum ferritin levels increased after administration of low-dose intravenous bevacizumab and were maintained. The frequency of red blood cell transfusions decreased. However, the hemoglobin level again decreased, and blood transfusions were required after the fourth bevacizumab cycle.

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