Resumen
La concepción de lo que entendemos como púrpura trombocitopénica inmune (PTI) ha cambiado en los últimos 10 años. De ser una patología exclusivamente hemorrágica donde el objetivo del tratamiento se basaba solo en conseguir un recuento de plaquetas ¨seguro¨ (mayor a 50.000 /uL) hoy concebimos a la PTI como una verdadera entidad trombo-hemorrágica. El nuevo concepto de la enfermedad se explica al entender mejor su fisiopatología que incluye fenómenos de trombo-inflamación, activación inmune, del complemento y de la hemostasia y un aumento de eventos trombóticos. También intervienen en este fenómeno los medicamentos o procedimientos como la esplenectomía, que aumentan el riesgo de trombosis, en nuestro empecinado intento de normalizar el recuento de plaquetas. Esto, en una enfermedad con un pico de incidencia en la población añosa, que tiene aumentados los factores de riesgo cardiovascular, explica los fenómenos trombóticos de la PTI. Por eso se recomienda individualizar el tratamiento en esta patología considerando no solo los factores potenciadores de sangrado y de trombosis, también la calidad de vida del paciente, los síntomas generales como astenia y decaimiento, la toxicidad a corto y largo plazo de la terapia instituida y el acceso al tratamiento, según los recursos regionales de cada institución. Hoy nuevos agentes para el tratamiento de la PTI están disponibles en nuestro medio como el fostamatinib, con un mecanismo de acción diferente y menor efecto inmunosupresor, pero especialmente con menor riesgo trombótico. Y próximamente tendremos acceso al rilzabrutinib, un inhibidor de la Bruton quinasa con potente efecto en múltiples sistemas potenciadores de inflamación de la PTI. En esta revisión evaluaremos el rol de estos nuevos agentes y su lugar en pacientes considerados de alto riesgo trombótico, pero con plaquetopenia extrema por PTI.
Citas
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