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.)

المؤلفون: Blanes, M., Lorenzo, J. I., Ribas, P., Jiménez, A., González, J. D., Cejalvo, M. J., Solano, C., Alegre, A., de la Rubia, Javier

المصدر: Annals of Hematology; Aug2019, Vol. 98 Issue 8, p2013-2015, 3p

مصطلحات موضوعية: MULTIPLE myeloma, BUSULFAN, MELPHALAN, DRUG side effects, STEM cell transplantation

المؤلفون: Alvarez-Larrán, Alberto, del Río-Garma, Julio, Pujol, Misericòrdia, de la Rubia, Javier, Hernández-Jodra, Manuel, Borrell, Montserrat, González-Porras, José R., García-Gala, José M., Viejo, Aurora, Enríquez, Lourdes, Arbona, Cristina, García-Erce, José A., Noblejas, Ana G., Pereira, Arturo

المصدر: Annals of Hematology; Oct2009, Vol. 88 Issue 10, p973-978, 6p, 2 Charts

مصطلحات موضوعية: THROMBOTIC thrombocytopenic purpura, PLASMA exchange (Therapeutics), BLOOD transfusion, HEMOLYTIC anemia, HEMOLYSIS & hemolysins, DISEASE relapse

مستخلص: The remission rate with plasma exchange (PE) in thrombotic thrombocytopenic purpura (TTP) exceeds 80%, but the disease relapses in up to 20–30% of the cases. Clinical characteristics and response to treatment of relapsed TTP are not well defined. The objective of the present study was to compare the clinical and biological characteristics at presentation and the response to treatment between de novo and relapsed TTP. For such purpose, a total of 102 episodes of idiopathic TTP (70 de novo and 32 relapses) included in a recent multicentric prospective cohort study were analysed. All patients were homogeneously treated with daily PE and costicosteroids. In comparison with de novo TTP, episodes of relapsed TTP showed a higher Hb level (median, 122 g/l versus 91 g/l, p < 0.001) and lower serum lactate dehydrogenase (2.2- versus 4.5-fold above the upper limit of normality, p < 0.001). Neurological symptoms and fever were less frequently observed in patients with relapsed TTP than in patients with de novo TTP. Patients with relapsed TTP needed fewer PE sessions (five versus ten, p = 0.02) and a smaller volume of plasma (221 ml/kg versus 468 ml/kg, p = 0.004) to achieve remission than those with de novo TTP. There was no significant difference in the rate of recrudescence under treatment, the need of complementary treatments or the frequency of refractoriness to PE therapy. In conclusion, relapsed TTP has a milder clinical profile and responds more easily to PE than de novo TTP. [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.)

المؤلفون: Cañigral, Carolina, Moscardó, Federico, Castro, Cristina, Pajares, Azucena, Lancharro, Aima, Solves, Pilar, de la Rubia, Javier, Carpio, Nelly, Sanz, Miguel A

المصدر: Annals of Hematology; Jun2008, Vol. 87 Issue 6, p1421-1422, 2p