- Artículo
- Fuente: Campus Sanofi
- 8 nov 2023
Morbilidad y Mortalidad a corto y largo plazo en PTTa
La Púrpura Trombocitopénica Trombótica (PTT) es una emergencia médica
La PTTa es una afección poco común de microangiopatía trombótica que puede tener consecuencias graves e incluso mortales para la salud, tanto a corto como a largo plazo. Por lo tanto, es fundamental que se realice un tratamiento inmediato.1-4
Consecuencias a corto plazo
Morbilidad6
![signos-y-sintomas-2](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/signos-y-sintomas-2.jpg/jcr:content/signos-y-sintomas-2.jpg)
Ictus
(10%)
![signos-y-sintomas-1](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/signos-y-sintomas-1.jpg/jcr:content/signos-y-sintomas-1.jpg)
Insuficiencia Cardíaca
(8%)
![signos-y-sintomas-4](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/signos-y-sintomas-4.jpg/jcr:content/signos-y-sintomas-4.jpg)
Síndrome coronario agudo
(6%)
![signos-y-sintomas-2](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/signos-y-sintomas-2.jpg/jcr:content/signos-y-sintomas-2.jpg)
Ictus
(10%)
![signos-y-sintomas-1](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/signos-y-sintomas-1.jpg/jcr:content/signos-y-sintomas-1.jpg)
Insuficiencia Cardíaca
(8%)
![signos-y-sintomas-4](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/signos-y-sintomas-4.jpg/jcr:content/signos-y-sintomas-4.jpg)
Síndrome coronario agudo
(6%)
Mortalidad
Sin tratamiento el desenlace fatal de la PTT es rápido, con una mortalidad del 90%2,5,6
![90porciento](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/90porciento.jpg/jcr:content/90porciento.jpg)
A pesar del tratamiento con recambio plasmático e inmunosupresión, los pacientes con PTTa mantienen un riesgo de complicaciones trombóticas y de mortalidad aguda de hasta un 8%-20%.
Consecuencias a largo plazo
Mortalidad
Se observaron mayores tasas de mortalidad en los pacientes que no alcanzaron una tasa de recuperación de plaquetas de 5x109/L al día en el tercer día de PEX13
Secuelas a largo plazo
![mortalidad.2023-12-20-14-17-55](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/mortalidad.2023-12-20-14-17-55.jpg/jcr:content/mortalidad.2023-12-20-14-17-55.jpg)
Aumento general de la mortalidad (recaídas de PTTa y enfermedad cardiovascular)14-16
![cerebro.2023-12-20-13-07-20](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/cerebro.2023-12-20-13-07-20.jpg/jcr:content/cerebro.2023-12-20-13-07-20.jpg)
5 veces más de riesgo de ictus incluso en remisión14
![neuro.2023-12-20-13-07-20](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/neuro.2023-12-20-13-07-20.jpg/jcr:content/neuro.2023-12-20-13-07-20.jpg)
Hasta 80% de aumento de depresión15,16
![dia.2023-12-20-13-07-20](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/dia.2023-12-20-13-07-20.jpg/jcr:content/dia.2023-12-20-13-07-20.jpg)
Reduce la calidad de vida incluso en remisión a largo plazo15,16
![prohibido.2023-12-20-14-16-07](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/prohibido.2023-12-20-14-16-07.jpg/jcr:content/prohibido.2023-12-20-14-16-07.jpg)
37 veces más de riesgo de sufrir lupus eritematoso sistémico y otras enfermedades autoinmunes15,16
![embarazo.2023-12-20-14-19-41](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/embarazo.2023-12-20-14-19-41.jpg/jcr:content/embarazo.2023-12-20-14-19-41.jpg)
Resultados adversos en el embarazo14
![tension.2023-12-20-13-30-35](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/tension.2023-12-20-13-30-35.jpg/jcr:content/tension.2023-12-20-13-30-35.jpg)
Hipertensión de novo
![rinones.2023-12-20-13-07-20](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/rinones.2023-12-20-13-07-20.jpg/jcr:content/rinones.2023-12-20-13-07-20.jpg)
Microalbuminuria16,17
![ptta.2023-12-20-14-29-07](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/ptta.2023-12-20-14-29-07.jpg/jcr:content/ptta.2023-12-20-14-29-07.jpg)
Deterioro cognitivo duradero15,16
![corazon.2023-12-20-13-07-20](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/corazon.2023-12-20-13-07-20.jpg/jcr:content/corazon.2023-12-20-13-07-20.jpg)
Accidente cardiovascular como principal causa de muerte entre los pacientes con PTTa15
Tres mecanismos clave impulsan la fisiopatología de la PTTa 2
![mortalidad](https://pro.campus.sanofi/.imaging/mte/portal/3840/dam/Portal/Spain/articulos/hematology/ptta-mortalidad/mortalidad-1.jpg/jcr:content/mortalidad-1.jpg)
Trombocitopenia grave
(plaquetas <30x109/L)
Anemia hemolítica microangiopática (MAHA, por sus siglas en inglés)
Caracterizada por la presencia de esquistocitos
Ausencia de causa identificable
Referencias
- Grall M, Azoulay E, Galicier L, et al. Thrombotic thrombocytopenic purpura misdiagnosed asautoimmune cytopenia: causes of diagnostic errors and consequence on outcome. Experience of the French Thrombotic Microangiopathies Reference Centre. Am J Hematol. 2017;92(4):381-387. doi:10.1002/ajh.24665
- Scully M, Hunt BJ, Benjamin S, et al; British Committee for Standards in Haematology. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012;158(3):323-335. doi: 10.1111/j.1365-2141.2012.09167.x
- Goel R, King KE, Takemoto CM, Ness PM, Tobian AAR. Prognostic riskstratified score for predicting mortality in hospitalized patients with thrombotic thrombocytopenic purpura: nationally representative data from 2007 to 2012. Transfusion. 2016;56(6):1451-1458. doi:10.1111/trf.13586
- Zheng XL, Vesely SK, Cataland SR, et al. ISTH guidelines for the diagnosis of thrombotic thrombocytopenic purpura. J Thromb Haemost. 2020;18(10):2486-2495 and suppl. doi:10.1111/jth. 15006
- Sayani FA, Abrams CS. How I treat refractory thrombotic thrombocytopenic purpura. Blood. 2015;125(25):3860–7.
- Kremer Hovinga JA, et al. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood. 2010; 115(8):1500–11.
- Terrell DR, et al. The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency. J Thromb Haemost. 2005 Jul;3(7):1432-6
- Miller DP, et al. Incidence of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome. Epidemiology. 2004 Mar;15(2):208-15
- Joly BS, Coppo P, Veyradier A. Thrombotic thrombocytopenic purpura. Blood. 2017;129(21):2836-2846.
- Azoulay E, et al. Expert statement on the ICU management of patients with thrombotic thrombocytopenic purpura. Intensive Care Med. 2019;45(11):1518-1539.
- Gallan AJ, Chang A. A new paradigm for renal thrombotic microangiopathy. Semin Diagn Pathol. 2020;37(3):121-126.
- Long B, et al. Evaluation and Management of Thrombotic Thrombocytopenic Purpura in the Emergency Department. J Emerg Med. 2021 Dec;61(6):674-682.
- Liu C, Kallogjeri D, Dynis M, Grossman BJ. Platelet recovery rate during plasma exchange predicts early and late responses in patients with thrombotic thrombocytopenic purpura. Transfusion. 2013;53(5):1096-1107. doi: 10.1111/j.1537-2995.2012. 03857.x
- Sukumar S, Brodsky M, Hussain S, et al. Cardiovascular Disease Is a Leading Cause of Death in Thrombotic Thrombocytopenic Purpura (TTP) Survivors. Blood. 2020;136(Suppl 1):22-3.
- Sukumar S, Gavriilaki E, Chaturvedi S. Updates on thrombotic thrombocytopenic purpura: Recent developments in pathogenesis, treatment and survivorship, Thrombosis Update. 2021;5:100062.
- George JN. TTP: long-term outcomes following recovery. Hematology Am Soc Hematol Educ Program. 2018;2018(1):548-52.
- Little DJ, Reese JA, Vesely SH, et al. Increased Urinary Albumin Excretion Following Recovery From Thrombotic Thrombocytopenic Purpura Due to Acquired ADAMTS13 Deficiency. Am J Kidney Dis. 2014;64(2):317-8.
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