Presenting ADAMTS13 antibody and antigen levels predict prognosis in immune-mediated thrombotic thrombocytopenic purpura
| Autor: | Oliver Chapman, William Lester, Debra Ellis, K. Douglas, John-Paul Westwood, Sylvia Benjamin, Tanya Cranfield, Chiara Vendramin, Katy Langley, Tina T. Biss, J. J. van Veen, Karen Vanhoorelbeke, Lynn Manson, Ferras Alwan, Mari Thomas, Nichola Cooper, Quentin A. Hill, Steve Austin, Tina Dutt, Richard Gooding, Amanda Clark, Marie Scully, Vickie McDonald, Henry G. Watson, William Thomas, Keith Sibson |
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| Rok vydání: | 2017 |
| Předmět: |
Adult
Male Adolescent Immunology Thrombotic thrombocytopenic purpura ADAMTS13 Protein 030204 cardiovascular system & hematology Biochemistry Disease-Free Survival 03 medical and health sciences 0302 clinical medicine Immune system Antigen hemic and lymphatic diseases medicine Humans Child Prospective cohort study Survival rate Aged Autoantibodies Aged 80 and over Purpura Thrombotic Thrombocytopenic biology business.industry Cell Biology Hematology Middle Aged medicine.disease ADAMTS13 Survival Rate Quartile Immunoglobulin G biology.protein Female Antibody business 030215 immunology |
| Zdroj: | Blood. 130:466-471 |
| ISSN: | 1528-0020 0006-4971 |
| DOI: | 10.1182/blood-2016-12-758656 |
| Popis: | 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 77%) had a mortality of 16.9% compared with 5.0% for the lowest quartile (antibody 11%) (P = .005). The synergistic effect of anti-ADAMTS13 IgG antibody in the upper quartile and ADAMTS13 antigen in the lowest quartile had the highest mortality of 27.3%. We conclude that both anti-ADAMTS13 IgG antibody and ADAMTS13 antigen levels correlate with outcome in TTP with increased cardiac and neurological involvement and increased mortality. |
| Databáze: | OpenAIRE |
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