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]

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المؤلفون: Pascual‐Izquierdo, Cristina, del Rio‐Garma, Julio, de la Rubia, Javier, Viejo, Aurora, Mingot, Eva, Cid, Joan, Solanich, Xavier, Fernández‐Sojo, Jesús, Martín‐Sánchez, Jesús, Hernández, Luis, García‐Gala, José María, Alonso, Nieves, González, Victoria, Oliva, Ana, Gómez‐Seguí, Inés, Goterris, Rosa, Guerra, Luisa, García‐Candel, Faustino, Fernández‐Docampo, Marta, Antelo, María Luisa

المصدر: Journal of Clinical Apheresis; Aug2021, Vol. 36 Issue 4, p563-573, 11p

مصطلحات موضوعية: THROMBOTIC thrombocytopenic purpura, DIAGNOSIS, IDIOPATHIC thrombocytopenic purpura, PROGNOSIS, DISEASE management, DISEASE incidence, THERAPEUTIC complications

مصطلحات جغرافية: SPAIN

مستخلص: Background: Immune‐mediated thrombotic thrombocytopenic purpura (iTTP) is a rare disease characterized by the presence of anti‐ADAMTS13 autoantibodies. Achieving accurate information on incidence and customary disease management is important to provide appropriate diagnostic and therapeutic resources. The aim of this study was to determine the incidence and outcomes of iTTP in Spain. Study design and methods: A cross‐sectional survey was carried out among Spanish hospitals, focused on iTTP patients ≥16 years old attended between 2015 and 2017, and those at follow‐up before that interval. Incidence, prevalence, mortality, refractoriness, exacerbations, treatment complications, relapses, and sequelae were estimated. Results: Forty‐two hospitals covering roughly 20 million inhabitants answered the survey and reported 203 episodes (138 newly‐diagnosed and 65 relapses), of which 193 (95.1%) were treated. Incidence was 2.67 (95% CI 1.90‐3.45) patients per million inhabitants per year and prevalence 21.44 (95% CI% 19.10‐23.73) patients per million inhabitants. At diagnosis, ADAMTS13 activity and anti‐ADAMTS13 autoantibody were measured in 97% and 84.3% of reported episodes, respectively. Fifteen patients (7.4%) died as a direct consequence of iTTP, 6 of them before receiving any iTTP‐specific treatment. Thirty‐one (16.1%) of the 193 treated episodes were refractory to plasma exchange and corticosteroids, and 51 (26.4%) suffered at least one exacerbation. Conclusion: iTTP incidence and prevalence were somewhat higher than those documented in neighboring countries. Together with data on treatments and outcomes, this information will allow us to better estimate what is needed to improve diagnosis and prognosis of iTTP patients in Spain. [ABSTRACT FROM AUTHOR]

: Copyright of Journal of Clinical Apheresis is the property of Wiley-Blackwell 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: 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: del Río-Garma, Julio, Alvarez-Larrán, Alberto, Martínez, Clara, Muncunill, Josep, Castell, Dolors, de la Rubia, Javier, Zamora, Concepción, Corral, Mercedes, Viejo, Aurora, Peña, Francisco, Rodríguez-Vicente, Pilar, Contreras, Enric, Arbona, Cristina, Ramírez, Consuelo, Garcia-Erce, José A., Alegre, Adrián, Mateo, Jos, Pereira, Arturo

المصدر: British Journal of Haematology; Oct2008, Vol. 143 Issue 1, p39-45, 7p, 3 Charts

مصطلحات موضوعية: THROMBOTIC thrombocytopenic purpura, HEMOLYTIC anemia, PLASMA exchange (Therapeutics), BLOOD transfusion, COMMUNICABLE diseases, BLOOD plasma

مستخلص: Plasma exchange (PE) with plasma infusion is the treatment of choice for thrombotic thrombocytopenic purpura (TTP) but doubts remain as to whether all kinds of plasma are equally effective. A multicentric cohort study was conducted to compare methylene blue-photoinactivated plasma (MBPIP) with quarantine fresh frozen plasma (qFFP) in the treatment of TTP. One hundred and two episodes of idiopathic TTP were included; MBPIP was used in 63 and qFFP in 39. The treatment schedule consisted of daily PE and costicosteroids, and the main end-point was remission status on day 8. Patients treated with MBPIP required more PEs (median: 11 vs. 5, P = 0·002) and a larger volume of plasma (median: 485 ml/kg vs. 216 ml/kg, P = 0·007) to achieve a remission, and presented more recrudescences while on PE therapy (29 of 63 vs. 8 of 39, P = 0·02) than those receiving qFFP. After adjustment for possible confounding factors, the use of MBPIP was associated with a lower likelihood of remission on day 8 [Odds ratio (OR): 0·17; 95% confidence interval (CI): 0·06–0·47] and a higher risk of recrudescence while on treatment (OR: 4·2; 95% CI: 1·6–10·8). In conclusion, MBPIP is less effective than qFFP in the treatment of TTP. [ABSTRACT FROM AUTHOR]

: Copyright of British Journal of Haematology is the property of Wiley-Blackwell 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: de la Rubia, Javier¹, López, Aurelio¹, Arriaga, Francisco¹, Cid, Ana Rosa¹, Vicente, Ana Isabel¹, Marty, Maria Luisa¹, Sanz, Miguel A.¹

المصدر: Acta Haematologica. 1999, Vol. 102 Issue 1, p12-16. 5p.

مصطلحات موضوعية: *PLASMA exchange (Therapeutics), *STEROIDS, *THERAPEUTICS, *THROMBOTIC thrombocytopenic purpura, *THROMBOPENIC purpura

مستخلص: We describe our experience in the management of 11 consecutive patients with thrombotic thrombocytopenic purpura (TTP) treated with a combined therapy of plasma exchange (PE) and steroids. Nine patients (82%) achieved complete remission (CR) after a median of 6 rounds of PE (range 2–22). There were 3 early relapses managed in the same way as the initial episode. One patient relapsed 23 months after diagnosis achieving CR with standard therapy; another patient suffered several relapses, and splenectomy was performed after the last one. Three patients died, 2 of them with resistant disease 9 and 38 days after diagnosis, and the remaining one died due to AIDS-related complications while he was in CR. Eight patients are alive in CR with a median follow-up of 38 months (range 8–74). The combination of PE and steroids is a well-tolerated and effective treatment of TTP, but improvements in therapy are needed to manage refractory patients. [ABSTRACT FROM AUTHOR]

المؤلفون: de la Rubia, Javier¹, Arriaga, Francisco¹, Linares, Dolores¹, Larrea, Luis¹, Carpio, Nelly¹, Marty, María L.¹, Sanz, Miguel A.¹

المصدر: British Journal of Haematology. Sep2001, Vol. 114 Issue 3, p721-723. 3p. 2 Charts.

مصطلحات موضوعية: *THROMBOTIC thrombocytopenic purpura treatment, *PLASMA exchange (Therapeutics)

مستخلص: Twenty patients with thrombotic thrombocytopenic purpura (TTP) underwent plasma exchange using either standard fresh-frozen plasma (Group A, n = 13) or methylene blue-treated plasma (Group B, n = 7). Both groups presented similar characteristics except that bilirubin values were higher in Group A (P < 0·05). The complete remission rate was higher in Group A than B (69% versus 57%). The mean number of procedures was higher in Group B (21 ± 7 versus 11 ± 3, P < 0·01) and the mean duration of hospitalization was also longer (37 ± 12 d versus 22 ± 11 d; P < 0·01). Our study shows that the use of methylene blue-treated fresh-frozen plasma to treat TTP is associated with a higher number of plasma exchanges and greater transfusion requirements without improving clinical results. [ABSTRACT FROM AUTHOR]