How targeted therapy disrupts the treatment paradigm for acquired TTP: the risks, benefits, and unknowns

被引:35
作者
Mazepa, Marshall A. [1 ]
Masias, Camila [2 ]
Chaturvedi, Shruti [3 ]
机构
[1] Univ Minnesota, Dept Med, Box 736 UMHC, Minneapolis, MN 55455 USA
[2] Ohio State Univ, Dept Med, Div Hematol, Columbus, OH 43210 USA
[3] Johns Hopkins Univ, Sch Med, Dept Med, Div Hematol, 720 Rutland Ave,Ross Res Bldg,Room 1025, Baltimore, MD 21205 USA
关键词
THROMBOTIC THROMBOCYTOPENIC PURPURA; PLASMA-EXCHANGE; ANTIBODY DEPLETION; ADAMTS13; ACTIVITY; RITUXIMAB; MICROANGIOPATHIES; BORTEZOMIB; EXPERIENCE; REMISSION; SAFETY;
D O I
10.1182/blood.2019000954
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Insights into immune-mediated thrombotic thrombocytopenic purpura (iTTP) pathophysiology have led to novel targeted therapies. Immunomodulatory strategies target anti-ADAMTS13 antibodies: rituximab is effective in inducing responses in refractory/relapsed TTP and increasing relapse-free survival; caplacizumab targets the von Willebrand factor-platelet interaction to hasten platelet count recovery and reduce mortality and TTP-related ischemic events. Bortezomib and recombinant ADAMTS13 are under investigation. This review examines how targeted therapies are disrupting current treatment paradigms to improve outcomes of iTTP.
引用
收藏
页码:415 / 420
页数:6
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