Association between N-acetylcysteine treatment and in-hospital mortality in adult patients with acquired thrombotic thrombocytopenic purpura: a cohort study
Abstract Acquired thrombotic thrombocytopenic purpura (aTTP) is a fatal hematologic disease. Despite the currently high standards of care, some patients who develop refractory or recurrent disease still have a poor prognosis. Although N-acetylcysteine (NAC) is recommended for the treatment of aTTP, its use in aTTP treatment is still controversial. We aimed to evaluate the association of NAC with mortality in patients with aTTP. This was a retrospective cohort study of patients with aTTP with in-hospital mortality as the primary outcome and time to platelet recovery and neurological recovery as secondary outcomes. We used multifactorial COX regression analysis to check for an association of NAC with mortality. Moreover, we performed a sensitivity analysis check the stability of our results. Finally, 89 patients with aTTP were enrolled. After adjusting for potential confounders, we found NAC to be associated with 75% lower in-hospital mortality (HR = 0.25, 95% CI = 0.1–0.64). The results of sensitivity analyses performed remained stable as the risk of in-hospital mortality in patients reduced in patients with comorbid neurological symptoms (HR = 0.23, 95% CI = 0.06–0.89). However, NAC use did not affect the time to platelet recovery (HR = 1.19, 95% CI = 0.57–2.5) or neurological recovery (HR = 0.32, 95% CI = 0.08–1.25) in patients with aTTP. NAC treatment reduces in-hospital mortality in patients with aTTP but does not shorten the time to platelet recovery or neurological recovery..
| Medienart: |
Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:102 |
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| Enthalten in: |
Annals of hematology - 102(2023), 8 vom: 14. Juni, Seite 2257-2265 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Li, Jing [VerfasserIn] |
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| Links: |
Volltext [lizenzpflichtig] |
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| Themen: |
Acquired thrombotic thrombocytopenic purpura (aTTP) |
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| Anmerkungen: |
© The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2023. 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/s00277-023-05295-2 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
OLC2144440182 |
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| 520 | |a Abstract Acquired thrombotic thrombocytopenic purpura (aTTP) is a fatal hematologic disease. Despite the currently high standards of care, some patients who develop refractory or recurrent disease still have a poor prognosis. Although N-acetylcysteine (NAC) is recommended for the treatment of aTTP, its use in aTTP treatment is still controversial. We aimed to evaluate the association of NAC with mortality in patients with aTTP. This was a retrospective cohort study of patients with aTTP with in-hospital mortality as the primary outcome and time to platelet recovery and neurological recovery as secondary outcomes. We used multifactorial COX regression analysis to check for an association of NAC with mortality. Moreover, we performed a sensitivity analysis check the stability of our results. Finally, 89 patients with aTTP were enrolled. After adjusting for potential confounders, we found NAC to be associated with 75% lower in-hospital mortality (HR = 0.25, 95% CI = 0.1–0.64). The results of sensitivity analyses performed remained stable as the risk of in-hospital mortality in patients reduced in patients with comorbid neurological symptoms (HR = 0.23, 95% CI = 0.06–0.89). However, NAC use did not affect the time to platelet recovery (HR = 1.19, 95% CI = 0.57–2.5) or neurological recovery (HR = 0.32, 95% CI = 0.08–1.25) in patients with aTTP. NAC treatment reduces in-hospital mortality in patients with aTTP but does not shorten the time to platelet recovery or neurological recovery. | ||
| 650 | 4 | |a Acquired thrombotic thrombocytopenic purpura (aTTP) | |
| 650 | 4 | |a N-acetylcysteine (NAC) | |
| 650 | 4 | |a In-hospital mortality | |
| 650 | 4 | |a Time to platelet recovery | |
| 650 | 4 | |a Time to neurological recovery | |
| 700 | 1 | |a Zhang, You |4 aut | |
| 700 | 1 | |a Kong, Danqing |4 aut | |
| 700 | 1 | |a Su, Jian |4 aut | |
| 700 | 1 | |a Wei, Yao |4 aut | |
| 700 | 1 | |a Liu, Xueke |4 aut | |
| 700 | 1 | |a Lu, Shiqi |4 aut | |
| 700 | 1 | |a Wang, Jun |4 aut | |
| 700 | 1 | |a Huang, Fang |4 aut | |
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