Comparison of clinical characteristics of first-episode thrombotic thrombocytopenic purpura and TTP-like syndrome: a retrospective cohort study in a level I hematology center in China
Abstract Comparing the characteristics of thrombotic thrombocytopenic purpura (TTP) and TTP-like syndrome patients at admission will allow early differentiation of TTP from TTP-like syndrome and help tailor initial treatment. The medical records of 78 patients with suspected TTP in the Emergency Department of Peking University People’s Hospital in the past 5 years were retrospectively analyzed and divided into TTP and TTP-like syndrome groups based on ADAMTS13 activity and ADAMTS13 antibody titer. There were 25 and 53 patients in the TTP group and the TTP-like syndrome group, respectively. The neutrophil-to-lymphocyte ratio (P = 0.025) was tremendously higher, and albumin (P = 0.002) was lower in the TTP-like syndrome group, indicating a more severe inflammation. Compared with the TTP-like syndrome group, the TTP group had an approximately two-fold to three-fold higher prevalence of central nervous system dysfunction (P < 0.001). Also, hemolysis was more substantial in the TTP group as evidenced by higher schistocytes (P < 0.001), reticulocyte (P < 0.001), total bilirubin (P = 0.002), indirect bilirubin (P < 0.001), lactate dehydrogenase (P = 0.007) and cell-free hemoglobin (P < 0.001), simultaneously lower platelet (P < 0.001), haptoglobin (P = 0.044), and ADAMTS13 activity (P < 0.001). The Kaplan–Meier survival analysis showed that the TTP group significantly predicted poor prognosis (log-rank test: $ X^{2} $ = 5.368, P = 0.021). TTP and TTP-like syndrome are two kinds of distinct phenotypes with different hemolysis statuses and illustrated differentiated inflammatory reactions, target organ damage (TOD), and the clinical outcome..
| Medienart: |
Artikel |
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| Erscheinungsjahr: |
2022 |
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| Erschienen: |
2022 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:102 |
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| Enthalten in: |
Annals of hematology - 102(2022), 1 vom: 17. Nov., Seite 55-62 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Dong, Guiying [VerfasserIn] |
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| Links: |
Volltext [lizenzpflichtig] |
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| Themen: |
In-hospital mortality |
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| Anmerkungen: |
© The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2022. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. |
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| doi: |
10.1007/s00277-022-05030-3 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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OLC2080209469 |
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| 520 | |a Abstract Comparing the characteristics of thrombotic thrombocytopenic purpura (TTP) and TTP-like syndrome patients at admission will allow early differentiation of TTP from TTP-like syndrome and help tailor initial treatment. The medical records of 78 patients with suspected TTP in the Emergency Department of Peking University People’s Hospital in the past 5 years were retrospectively analyzed and divided into TTP and TTP-like syndrome groups based on ADAMTS13 activity and ADAMTS13 antibody titer. There were 25 and 53 patients in the TTP group and the TTP-like syndrome group, respectively. The neutrophil-to-lymphocyte ratio (P = 0.025) was tremendously higher, and albumin (P = 0.002) was lower in the TTP-like syndrome group, indicating a more severe inflammation. Compared with the TTP-like syndrome group, the TTP group had an approximately two-fold to three-fold higher prevalence of central nervous system dysfunction (P < 0.001). Also, hemolysis was more substantial in the TTP group as evidenced by higher schistocytes (P < 0.001), reticulocyte (P < 0.001), total bilirubin (P = 0.002), indirect bilirubin (P < 0.001), lactate dehydrogenase (P = 0.007) and cell-free hemoglobin (P < 0.001), simultaneously lower platelet (P < 0.001), haptoglobin (P = 0.044), and ADAMTS13 activity (P < 0.001). The Kaplan–Meier survival analysis showed that the TTP group significantly predicted poor prognosis (log-rank test: $ X^{2} $ = 5.368, P = 0.021). TTP and TTP-like syndrome are two kinds of distinct phenotypes with different hemolysis statuses and illustrated differentiated inflammatory reactions, target organ damage (TOD), and the clinical outcome. | ||
| 650 | 4 | |a Thrombotic thrombocytopenic purpura | |
| 650 | 4 | |a TTP-like syndrome | |
| 650 | 4 | |a In-hospital mortality | |
| 700 | 1 | |a Shi, Fang-e |4 aut | |
| 700 | 1 | |a Yu, Jianbo |4 aut | |
| 700 | 1 | |a Zhu, Jihong |4 aut | |
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