Presenting ADAMTS13 antibody and antigen levels predict prognosis in immune-mediated thrombotic thrombocytopenic purpura

• Published in: Blood 2017-07, Vol.130 (4), p.466-471
• Main Authors: Alwan, Ferras, Vendramin, Chiara, Vanhoorelbeke, Karen, Langley, Katy, McDonald, Vickie, Austin, Steve, Clark, Amanda, Lester, William, Gooding, Richard, Biss, Tina, Dutt, Tina, Cooper, Nichola, Chapman, Oliver, Cranfield, Tanya, Douglas, Kenny, Watson, H.G., van Veen, J.J., Sibson, Keith, Thomas, William, Manson, Lynn, Hill, Quentin A., Benjamin, Sylvia, Ellis, Debra, Westwood, John-Paul, Thomas, Mari, Scully, Marie
• Format: Article
• Language: English
• Subjects: ADAMTS13 Protein - blood; ADAMTS13 Protein - immunology; Adolescent; Adult; Aged; Aged, 80 and over; Autoantibodies - blood; Autoantibodies - immunology; Child; Disease-Free Survival; Female; Humans; Immunoglobulin G - blood; Immunoglobulin G - immunology; Male; Middle Aged; Purpura, Thrombotic Thrombocytopenic - blood; Purpura, Thrombotic Thrombocytopenic - immunology; Purpura, Thrombotic Thrombocytopenic - mortality; Survival Rate
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood-2016-12-758656

Immune-mediated thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder caused by antibodies against ADAMTS13. From the United Kingdom TTP registry, we undertook a prospective study investigating the impact of the presenting anti-ADAMTS13 IgG antibody and ADAMTS13 antigen on mortality. A total of 312 episodes involving 292 patients over 87 months were included; 68% were female, median age 46 (range, 11-88 years), and median presenting ADAMTS13 of