Hereditary hemorrhagic telangiectasia: systemic therapies, guidelines, and an evolving standard of care

被引:52
作者
Al-Samkari, Hanny [1 ]
机构
[1] Harvard Med Sch, Massachusetts Gen Hosp, Div Hematol, Boston, MA 02115 USA
关键词
ENDOTHELIAL GROWTH-FACTOR; EPISTAXIS SEVERITY SCORE; VASCULAR MALFORMATIONS; IRON-DEFICIENCY; TRANEXAMIC ACID; DOUBLE-BLIND; FACTOR-VIII; BEVACIZUMAB; THALIDOMIDE; ANEMIA;
D O I
10.1182/blood.2020008739
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Hereditary hemorrhagic telangiectasia (HHT) management is evolving because of the emergence and development of antiangiogenic therapies to eliminate bleeding telangiectasias and achieve hemostasis. This progress is reflected in recent clinical recommendations published in the Second International Guidelines for the Diagnosis and Treatment of HHT, in which systemic therapies including antiangiogenics and antifibrinolytics are now recommended as standard treatment options for bleeding. This review highlights the new recommendations especially relevant to hematologists in managing bleeding, anticoagulation, and anemia in patients with HHT.
引用
收藏
页码:888 / 895
页数:8
相关论文
共 77 条
[1]  
AASSAR OS, 1991, LARYNGOSCOPE, V101, P977
[2]   Systemic bevacizumab for the treatment of chronic bleeding in hereditary haemorrhagic telangiectasia [J].
Al-Samkari, H. ;
Kritharis, A. ;
Rodriguez-Lopez, J. M. ;
Kuter, D. J. .
JOURNAL OF INTERNAL MEDICINE, 2019, 285 (02) :223-231
[3]   An international, multicenter study of intravenous bevacizumab for bleeding in hereditary hemorrhagic telangiectasia: the InHIBIT-Bleed study [J].
Al-Samkari, Hanny ;
Kasthuri, Raj S. ;
Parambil, Joseph G. ;
Albitar, Hasan A. ;
Almodallal, Yahya A. ;
Vazquez, Carolina ;
Serra, Marcelo M. ;
Dupuis-Girod, Sophie ;
Wilsen, Craig B. ;
McWilliams, Justin P. ;
Fountain, Evan H. ;
Gossage, James R. ;
Weiss, Clifford R. ;
Latif, Muhammad A. ;
Issachar, Assaf ;
Mei-Zahav, Meir ;
Meek, Mary E. ;
Conrad, Miles ;
Rodriguez-Lopez, Josanna ;
Kuter, David J. ;
Iyer, Vivek N. .
HAEMATOLOGICA, 2021, 106 (08) :2161-2169
[4]   An international survey to evaluate systemic bevacizumab for chronic bleeding in hereditary haemorrhagic telangiectasia [J].
Al-Samkari, Hanny ;
Albitar, Hasan A. ;
Olitsky, Scott E. ;
Clancy, Marianne S. ;
Iyer, Vivek N. .
HAEMOPHILIA, 2020, 26 (06) :1038-1045
[5]   Current and emerging pharmacotherapies for hereditary hemorrhagic telangiectasia [J].
Albinana, Virginia ;
Recio-Poveda, Lucia ;
Zarrabeitia, Roberto ;
Botella, Luisa M. .
EXPERT OPINION ON ORPHAN DRUGS, 2017, 5 (08) :665-675
[6]   Immunosuppressor FK506 Increases Endoglin and Activin Receptor-Like Kinase 1 Expression and Modulates Transforming Growth Factor-β1 Signaling in Endothelial Cells [J].
Albinana, Virginia ;
Sanz-Rodriguez, Francisco ;
Recio-Poveda, Lucia ;
Bernabeu, Carmelo ;
Botella, Luisa M. .
MOLECULAR PHARMACOLOGY, 2011, 79 (05) :833-843
[7]  
Albitar HAH, 2020, MAYO CLIN PROC, V95, P1604, DOI 10.1016/j.mayocp.2020.03.001
[8]   Thalidomide for the Management of Bleeding Episodes in Patients with Hereditary Hemorrhagic Telangiectasia: Effects on Epistaxis Severity Score and Quality of Life [J].
Baysal, Mehmet ;
Umit, Elif G. ;
Kirkizlar, Hakki Onur ;
Ozdover, Ali Caner ;
Demir, Ahmet Muzaffer .
TURKISH JOURNAL OF HEMATOLOGY, 2019, 36 (01) :43-47
[9]   Integration of clinical parameters, genotype and epistaxis severity score to guide treatment for hereditary hemorrhagic telangiectasia associated bleeding [J].
Beckman, Joan D. ;
Li, Quefeng ;
Hester, Samuel T. ;
Leitner, Ofri ;
Smith, Karen L. ;
Kasthuri, Raj S. .
ORPHANET JOURNAL OF RARE DISEASES, 2020, 15 (01)
[10]   Safety of thalidomide and bevacizumab in patients with hereditary hemorrhagic telangiectasia [J].
Buscarini, Elisabetta ;
Maria Botella, Luisa ;
Geisthoff, Urban ;
Kjeldsen, Anette D. ;
Mager, Hans Jurgen ;
Pagella, Fabio ;
Suppressa, Patrizia ;
Zarrabeitia, Roberto ;
Dupuis-Girod, Sophie ;
Shovlin, Claire L. .
ORPHANET JOURNAL OF RARE DISEASES, 2019, 14 (1)