ADAMTS13 testing update: Focus on laboratory aspects of difficult thrombotic thrombocytopenic purpura diagnoses and effects of new therapies

被引:8
作者
Smock, Kristi J. [1 ]
机构
[1] Univ Utah, Dept Pathol, ARUP Labs, Salt Lake City, UT USA
关键词
ADAMTS13; caplacizumab; laboratory testing; thrombotic thrombocytopenic purpura; VON-WILLEBRAND-FACTOR; ASSAYS; MICROANGIOPATHIES; VALIDATION; ANTIBODIES; INHIBITOR; REMISSION; ANTIGEN; UTILITY; SCORE;
D O I
10.1111/ijlh.13557
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
TTP is a life-threatening disorder diagnosed using a combination of clinical information and laboratory results. ADAMTS13 activity and antibody testing represent a major advance in the field, but results can sometimes be difficult to interpret due to technical aspects of the tests and characteristics of the causative antibodies in acquired TTP. Genetic testing for ADAMTS13 mutations is also now available to assist with the diagnosis of inherited TTP. This review will focus on ADAMTS13 testing and will highlight patient and laboratory aspects that can lead to diagnostic difficulty. The effects of TTP therapies on test results will also be discussed.
引用
收藏
页码:103 / 108
页数:6
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