التفاصيل البيبلوغرافية
| العنوان: |
Mortality in acquired thrombotic thrombocytopenic purpura in the pre-caplacizumab era. |
| المؤلفون: |
del Río-Garma, Julio, Bobillo, Sabela, de la Rubia, Javier, Pascual, Cristina, García-Candel, Faustino, García-Gala, Jose M., Gonzalez, Reyes, Abril, Laura, Vidan, Julia, Gomez, Maria Jesús, Peña, Francisco, Arbona, Cristina, Martín-Sanchez, Jesús, Moreno, Gemma, Romón, Iñigo, Viejo, Aurora, Oliva, Ana, Linares, Mónica, Salinas, Ramón, Pérez, Sonia |
| المصدر: |
Annals of Hematology; Jan2022, Vol. 101 Issue 1, p59-67, 9p |
| مصطلحات موضوعية: |
COMA, THROMBOTIC thrombocytopenic purpura, PROGNOSIS, PLATELET count, MORTALITY, EARLY death, PATIENTS' attitudes |
| مستخلص: |
Despite the effectiveness of plasma exchange (PEX) and immunosuppressants in the treatment of acquired thrombotic thrombocytopenic purpura (aTTP), a number of patients still die as a result of the disease. Whether caplacizumab could rescue these patients remains still unsettled. The objective of this study was to characterise mortality patterns and prognostic factors in the first episode of aTTP. We queried the Spanish TTP Registry for patients with a diagnosis of aTTP in their presenting episode who fulfilled complete clinical and follow-up data (n = 102). The patients were diagnosed between 2004 and 2018, and all were treated with daily PEX and corticosteroids. Clinical and laboratory data were analysed at diagnosis and during the treatment course. Eight patients (7.7%) died between 12 h and 36 days after presentation, and could be classified into three patterns: death before treatment, early death driven by acute cardiac or neurologic events, and late death due to unremitted aTTP. Stupor or coma at diagnosis and platelet count < 20 × 109 /L by the 6th treatment day were independently associated with increased risk of death. Stupor or coma at diagnosis and lack of response to PEX by the 6th day in patients experiencing the first episode of aTTP are strong predictors of mortality. These patients could be rescued by novel agents aimed at halting the microvascular thrombosis until adequate immunosuppression is achieved. [ABSTRACT FROM AUTHOR] |
|
Copyright of Annals of Hematology is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.) |
| قاعدة البيانات: |
Complementary Index |
