Estimaciones de la incidencia de las neoplasias hematológicas en España, 2021: estudio de la Red Española de Registros de Cáncer (REDECAN)

Sanvisens, Arantza; Ameijide, Alberto; Carulla, Marià; Marcos-Gragera, Rafael
doi:10.1016/j.medcli.2021.09.035
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que en 2017 se registraron alrededor de 1&#44;4 millones de casos incidentes de neoplasias hematol&#243;gicas en el mundo y el linfoma no hodgkiniano se situ&#243; entre uno de los 10 tipos de c&#225;ncer m&#225;s frecuentes y con mayor n&#250;mero absoluto de a&#241;os de vida perdidos<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">4</span></a>&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Desde la aparici&#243;n del virus SARS-COV-2 y las consecuentes reestructuraciones del sistema sanitario&#44; se ha observado una demora en la consulta m&#233;dica en general incluyendo a los pacientes que comienzan con un c&#225;ncer<a class="elsevierStyleCrossRef" href="#bib0175"><span class="elsevierStyleSup">5</span></a>&#46; Adem&#225;s&#44; la reticencia o miedo de los propios pacientes a acudir a los centros sanitarios tambi&#233;n pueden haber contribuido a esta demora&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">En general&#44; el pron&#243;stico del c&#225;ncer est&#225; muy ligado al momento del diagn&#243;stico y la intervenci&#243;n y&#44; en este sentido&#44; el impacto en el retraso diagn&#243;stico puede haber sido demoledor&#46; De hecho&#44; se estima que el exceso de mortalidad por c&#225;ncer derivado de esta pandemia llegue a su m&#225;ximo en los pr&#243;ximos 2 a&#241;os<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">6</span></a>&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">En el caso de las neoplasias hematol&#243;gicas&#44; los primeros datos evidencian un retraso que se traduce en un diagn&#243;stico de la enfermedad en fases m&#225;s avanzadas&#44; caracterizadas por ejemplo por una hiperleucocitosis en el caso de leucemias agudas o masas tumorales voluminosas en el caso de los linfomas<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">7</span></a>&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Por otro lado&#44; la propia infecci&#243;n por SARS-COV-2 puede haber tenido tambi&#233;n un fuerte impacto en el pron&#243;stico de los pacientes con c&#225;ncer que&#44; a causa de los tratamientos o la propia enfermedad&#44; suelen estar inmunodeprimidos&#46; Aunque algunos primeros estudios apuntan a que el pron&#243;stico de los pacientes con determinados tipos de neoplasia hematol&#243;gica y COVID-19 es comparable al de la poblaci&#243;n general que tambi&#233;n ha adquirido la infecci&#243;n<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">8</span></a>&#44; son necesarios estudios de base poblacional para establecer el impacto real de la pandemia en cada tipo de neoplasia&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Los estudios de predicciones poblacionales de casos de c&#225;ncer basados en el an&#225;lisis de series temporales se han demostrado eficaces para establecer la incidencia y mortalidad de esta enfermedad&#44; dado que el margen de error entre las cifras estimadas y las reales es insustancial para las pol&#237;ticas de gesti&#243;n sanitaria<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">9</span></a>&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Conocer el n&#250;mero de casos de c&#225;ncer esperados para 2021&#44; en un contexto de una pandemia sin precedentes&#44; es de vital utilidad para la planificaci&#243;n sanitaria de los pr&#243;ximos a&#241;os&#46; En este sentido&#44; nuestra hip&#243;tesis es que en el momento en que se disponga de las cifras reales de incidencia de 2021&#44; los resultados obtenidos en este estudio contribuir&#225;n a determinar el impacto de la COVID-19 en este grupo de enfermedades y a evaluar su repercusi&#243;n en la incidencia de los a&#241;os posteriores&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">El objetivo de este estudio es determinar los nuevos casos de los diferentes subtipos de neoplasia hematol&#243;gica en Espa&#241;a para 2021 en base a la incidencia poblacional observada en la Red Espa&#241;ola de Registros del C&#225;ncer &#40;REDECAN&#41; para 2002-2013&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">M&#233;todos</span><p id="par0050" class="elsevierStylePara elsevierViewall">Se incluyeron los datos de las neoplasias hematol&#243;gicas recogidas en los registros poblacionales que participan en REDECAN&#46; REDECAN actualmente dispone de una &#250;nica base de datos conjunta estandarizada para este tipo de neoplasias que incluye datos de 2002 a 2013&#46; En concreto&#44; se incluyeron datos de los registros de c&#225;ncer poblacionales &#40;RCP&#41; de &#193;lava&#44; Asturias &#40;2002-2010&#41;&#44; Bizkaia&#44; Castell&#243;n &#40;2004-2013&#41;&#44; Ciudad Real &#40;2004-2011&#41;&#44; Cuenca &#40;2002-2011&#41;&#44; Gipuzkoa&#44; Girona&#44; Gran Canaria&#44; Granada&#44; La Rioja&#44; Mallorca &#40;2003-2012&#41;&#44; Murcia &#40;2002-2010&#41;&#44; Navarra &#40;2002-2012&#41;&#44; Tarragona y Tenerife&#46; Estos registros cubren alrededor de un 26&#37; del total de poblaci&#243;n espa&#241;ola registrada en enero de 2013 &#40;12&#46;143&#46;157 de un total de 47&#46;129&#46;783&#41;<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">10</span></a>&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Los RCP utilizan distintas fuentes de informaci&#243;n y procedimientos para identificar los casos de neoplasias e incluirlos en el registro&#46; Las principales fuentes de informaci&#243;n de los registros que participan en REDECAN son los informes de anatom&#237;a patol&#243;gica que se complementan con los diagn&#243;sticos realizados en los laboratorios de hematolog&#237;a &#40;citometr&#237;a de flujo&#44; citogen&#233;tica y estudios de biolog&#237;a molecular&#41; siguiendo las recomendaciones internacionales <a class="elsevierStyleCrossRef" href="#bib0205"><span class="elsevierStyleSup">11</span></a>&#46; Para incluir un caso en el registro es necesario verificar la malignidad y especificar la base diagn&#243;stica&#44; as&#237; como registrar la informaci&#243;n en base a est&#225;ndares de codificaci&#243;n&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">En concreto&#44; para identificar los casos incidentes de neoplasias hematol&#243;gicas se incluyeron aquellos registros de tumor cuya definici&#243;n morfol&#243;gica estuviera comprendida entre los c&#243;digos 959 y 999 seg&#250;n la Clasificaci&#243;n Internacional de Enfermedades para Oncolog&#237;a-3&#46;<span class="elsevierStyleSup">a</span> edici&#243;n<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">12</span></a>&#44; independientemente de la localizaci&#243;n del tumor primario&#46; Los tumores se reagruparon siguiendo las directrices de la Organizaci&#243;n Mundial de la Salud de 2008 y 2017<a class="elsevierStyleCrossRefs" href="#bib0165"><span class="elsevierStyleSup">3&#44;13</span></a> y el proyecto HAEMACARE <a class="elsevierStyleCrossRef" href="#bib0220"><span class="elsevierStyleSup">14</span></a>&#44; seg&#250;n se muestra en la <a class="elsevierStyleCrossRef" href="#sec0050">tabla suplementaria 1</a>&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">Se definieron los indicadores de calidad en base a los est&#225;ndares de la <span class="elsevierStyleItalic">International Agency for Research on Cancer</span> &#40;Asociaci&#243;n Internacional para la Investigaci&#243;n del C&#225;ncer&#41;<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">15</span></a>&#46; En concreto&#44; los indicadores de calidad de los RCP mostraron que un 96&#44;7&#37; de casos incluidos fueron verificados microsc&#243;picamente&#44; un 5&#37; eran casos inespec&#237;ficos y un 1&#44;1&#37; casos notificados al registro &#250;nicamente en base a los certificados de defunci&#243;n&#46;</p><p id="par0070" class="elsevierStylePara elsevierViewall">Los datos poblacionales para el c&#225;lculo de las tasas de incidencia se obtuvieron de las cifras de poblaci&#243;n residente seg&#250;n a&#241;o&#44; provincia&#47;islas y sexo que proporciona el Instituto Nacional de Estad&#237;stica<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">10</span></a>&#46; Asimismo&#44; se obtuvieron los datos de proyecci&#243;n de poblaci&#243;n en Espa&#241;a para el a&#241;o 2021&#46;</p><p id="par0075" class="elsevierStylePara elsevierViewall">Este estudio se ha realizado utilizando datos anonimizados de los RCP participantes&#46; Por su parte&#44; los RCP cumplen con la normativa legal vigente en Espa&#241;a&#44; Ley Org&#225;nica 3&#47;2018 de Protecci&#243;n de Datos de Car&#225;cter Personal&#44; y normativa europea 2016&#47;679&#46; Los RCP pertenecen y cumplen con las regulaciones de la <span class="elsevierStyleItalic">International Association of Cancer Registries</span> &#40;Asociaci&#243;n Internacional de Registros de C&#225;ncer&#41; y la <span class="elsevierStyleItalic">International Agency for Research on Cancer</span>&#46; No se ha realizado ninguna intervenci&#243;n en seres humanos o en animales&#46; Para este tipo de estudios&#44; no se requiere consentimiento informado de los pacientes&#46;</p><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">An&#225;lisis estad&#237;stico</span><p id="par0080" class="elsevierStylePara elsevierViewall">Se realiz&#243; un an&#225;lisis descriptivo de los datos usando mediana y rango intercuart&#237;lico &#40;RIQ&#41; para las variables cuantitativas&#44; y frecuencias absolutas y porcentajes para las categ&#243;ricas&#46; Se calcularon las tasas de incidencia cruda &#40;TC&#41; y las tasas de incidencia ajustadas &#40;TA&#41; por edad en base a la poblaci&#243;n est&#225;ndar europea de 2013 <a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">16</span></a>&#44; y se expresaron por 100&#46;000 habitantes&#46; Se utilizaron modelos de regresi&#243;n de Poisson para analizar las tendencias de las tasas de incidencia y estimar el porcentaje anual de cambio &#40;PAC&#41; de cada subtipo de neoplasias&#46;</p><p id="par0085" class="elsevierStylePara elsevierViewall">Se estimaron las tasas espec&#237;ficas por edad para el a&#241;o 2021 aplicando los PAC del per&#237;odo 2002-2013&#46; La proyecci&#243;n del n&#250;mero de casos de neoplasias hematol&#243;gicas para 2021 se determin&#243; aplicando a la poblaci&#243;n espa&#241;ola de 2021 las tasas espec&#237;ficas por edad estimadas para ese mismo a&#241;o&#44; suponiendo una tendencia de la incidencia estable en el tiempo&#46; Los an&#225;lisis estad&#237;sticos se realizaron con el programa R versi&#243;n 3&#46;6&#46;1&#46;</p></span></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Resultados</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Caracter&#237;sticas de las neoplasias hematol&#243;gicas&#44; 2002-2013</span><p id="par0090" class="elsevierStylePara elsevierViewall">Entre 2002 y 2013 se registraron un total de 56&#46;777 neoplasias hematol&#243;gicas en los RCP participantes&#44; un 55&#37; correspond&#237;a a hombres y la mediana &#91;RIQ&#93; de edad al diagn&#243;stico fue de 69 &#91;54-78&#93; a&#241;os&#46; Un total de 39&#46;156 &#40;69&#37;&#41; de los tumores correspond&#237;an a neoplasias linfoides&#44; 17&#46;522 &#40;30&#44;8&#37;&#41; fueron neoplasias mieloides y 99 casos &#40;0&#44;2&#37;&#41; se registraron como histiocitosis&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">Con relaci&#243;n a las neoplasias linfoides&#44; un 55&#37; correspond&#237;a a hombres y la mediana &#91;RIQ&#93; de edad al diagn&#243;stico fue de 67 &#91;52-77&#93; a&#241;os&#46; La distribuci&#243;n de estas neoplasias seg&#250;n subtipo&#44; as&#237; como la mediana de edad de cada subtipo seg&#250;n sexo se pueden observar en la <a class="elsevierStyleCrossRef" href="#fig0005">figura 1</a>&#46; El mieloma m&#250;ltiple fue el subtipo de neoplasia linfoide m&#225;s frecuente &#40;19&#37;&#41;&#44; seguido por el linfoma difuso de c&#233;lulas B grandes &#40;17&#44;7&#37;&#41; y la leucemia linfoc&#237;tica cr&#243;nica&#47;linfoma linfoc&#237;tico de c&#233;lulas peque&#241;as &#40;15&#44;7&#37;&#41;&#44; este &#250;ltimo m&#225;s frecuente en hombres que en mujeres &#40;58&#44;4&#37; frente a 41&#44;6&#37;&#41;&#46; Globalmente&#44; la mediana de edad de las leucemias linfobl&#225;sticas fue de 18 &#91;RIQ&#58; 5-51&#93; a&#241;os&#46; En general&#44; la edad en el momento del diagn&#243;stico de las neoplasias linfoides fue mayor en mujeres que en hombres&#44; excepto en el caso del linfoma de Hodgkin&#44; micosis fungoides&#47;s&#237;ndrome de S&#233;zary y la leucemia de c&#233;lulas pilosas&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0100" class="elsevierStylePara elsevierViewall">Un 55&#37; de las neoplasias mieloides se diagnosticaron en hombres&#59; la mediana de edad en el momento del diagn&#243;stico fue de 73 &#91;RIQ&#58; 60-81&#93; a&#241;os&#46; Los subtipos espec&#237;ficos m&#225;s frecuentes fueron la trombocitemia esencial&#44; acumulando un 13&#44;7&#37; de los casos&#44; seguida de la leucemia mieloide cr&#243;nica &#40;7&#44;6&#37;&#41; y la policitemia vera &#40;6&#44;1&#37;&#41;&#44; si bien hay que tener en cuenta un elevado n&#250;mero de leucemia mieloide aguda y s&#237;ndromes mielodispl&#225;sicos &#40;SMD&#41; inespec&#237;ficos o no clasificados&#46; En la <a class="elsevierStyleCrossRef" href="#fig0010">figura 2</a> se muestra la distribuci&#243;n de las neoplasias mieloides seg&#250;n todos los subtipos analizados&#46; En general&#44; la edad en el momento del diagn&#243;stico de este tipo de neoplasias fue mayor en las mujeres &#40;<a class="elsevierStyleCrossRef" href="#fig0010">fig&#46; 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Incidencia&#44; tendencias y proyecciones 2021</span><p id="par0105" class="elsevierStylePara elsevierViewall">El n&#250;mero total de neoplasias hematol&#243;gicas estimadas para el a&#241;o 2021 en toda Espa&#241;a fue de 25&#46;879&#46; En las <a class="elsevierStyleCrossRefs" href="#tbl0005">tablas 1 y 2</a> se muestran las proyecciones de las neoplasias linfoides y mieloides seg&#250;n sexo&#44; respectivamente&#46; Las proyecciones totales se pueden consultar en la <a class="elsevierStyleCrossRef" href="#sec0050">tabla suplementaria 2</a>&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><p id="par0110" class="elsevierStylePara elsevierViewall">La TC de las neoplasias linfoides para el per&#237;odo 2002-2013 fue del 31&#44;2<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>100&#46;000 hab&#46; &#40;IC95&#37;&#58; 30&#44;9-31&#44;5&#41; y la TA 34&#44;2<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>100&#46;000 hab&#46; &#40;IC95&#37;&#58; 33&#44;9-34&#44;6&#41;&#46; El PAC de la incidencia observada fue de 0&#44;3 &#40;IC95&#37;&#58; &#8722;0&#44;1-0&#44;6&#41; proyectando una TC 36&#44;7<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>100&#46;000 hab&#46; &#40;IC95&#37;&#58;35&#44;9-37&#44;6&#41; y una TA de 35&#44;7<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>100&#46;000 hab&#46; &#40;IC95&#37;&#58; 34&#44;9-36&#44;5&#41; para 2021 en Espa&#241;a&#46; La <a class="elsevierStyleCrossRef" href="#fig0015">figura 3</a> muestra las TA anuales seg&#250;n sexo&#46; En concreto&#44; las proyecciones estimaron un total de 17&#46;385 &#40;IC95&#37;&#58; 16&#46;982-17&#46;788&#41; nuevos casos de neoplasias linfoides para 2021&#44; 9&#46;751 &#40;IC95&#37;&#58;9&#46;447-10&#46;055&#41; en hombres y 7&#46;634 &#40;IC95&#37;&#58;7&#46;370-7&#46;899&#41; en mujeres&#46; Las 3 neoplasias linfoides m&#225;s frecuentes proyectadas para 2021 fueron el linfoma difuso de c&#233;lulas B grandes &#40;19&#44;3&#37;&#41;&#44; el mieloma m&#250;ltiple &#40;19&#44;2&#37;&#41; y el linfoma folicular &#40;15&#44;0&#37;&#41;&#46; En la <a class="elsevierStyleCrossRef" href="#tbl0005">tabla 1</a> se muestran las proyecciones seg&#250;n sexo de las neoplasias linfoides en Espa&#241;a para 2021 seg&#250;n subtipo&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0115" class="elsevierStylePara elsevierViewall">En el caso de las neoplasias mieloides para el per&#237;odo 2002-2013&#44; la TC fue de 14<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>100&#46;000 hab&#46; &#40;IC95&#37;&#58;13&#44;8-14&#44;2&#41; y la TA 15&#44;5<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>100&#46;000 hab&#46; &#40;IC95&#37;&#58;15&#44;3-15&#44;7&#41;&#46; El PAC fue de 0&#44;7 &#40;IC95&#37;&#58; 0&#44;3-1&#44;2&#41; y la TC y TA estimadas para 2021 en Espa&#241;a fueron 17&#44;8<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>100&#46;000 hab&#46; &#40;IC95&#37;&#58;17&#44;2-18&#44;5&#41; y 17&#44;2<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>100&#46;000 hab&#46; &#40;IC95&#37;&#58;16&#44;6-17&#44;8&#41;&#44; respectivamente&#46; En total se proyectaron 8&#46;446 &#40;IC95&#37;&#58;8&#46;145-8&#46;747&#41; nuevos casos de neoplasias mieloides para 2021&#44; 4&#46;689 &#40;IC95&#37;&#58;4&#46;464-4&#46;914&#41; en hombres y 3&#46;757 &#40;IC95&#37;&#58;3&#46;557-3&#46;957&#41; en mujeres&#46; En la <a class="elsevierStyleCrossRef" href="#tbl0010">tabla 2</a> se pueden observar las estimaciones del n&#250;mero de casos y tasas de incidencia de las neoplasias mieloides en Espa&#241;a para 2021 seg&#250;n sexo y subtipo&#46; La trombocitemia esencial &#40;16&#44;8&#37;&#41;&#44; el SMD con displasia multilinaje &#40;8&#37;&#41; y la policitemia vera &#40;6&#44;6&#37;&#41; fueron los subtipos espec&#237;ficos m&#225;s frecuentes en las predicciones para 2021&#59; por otro lado&#44; la frecuencia de casos inespec&#237;ficos disminuy&#243; considerablemente respecto a la distribuci&#243;n observada en 2002-2013&#46;</p></span></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discusi&#243;n</span><p id="par0120" class="elsevierStylePara elsevierViewall">Los resultados de este estudio estiman que&#44; en 2021&#44; se deber&#237;an diagnosticar alrededor de 26&#46;000 nuevos casos de neoplasias hematol&#243;gicas en Espa&#241;a&#46; No obstante&#44; la situaci&#243;n pand&#233;mica en la cual nos hallamos actualmente&#44; as&#237; como los primeros resultados publicados sobre el impacto de la COVID-19 en la atenci&#243;n m&#233;dica en general&#44; y en el diagn&#243;stico del c&#225;ncer en particular&#44; indican que puede existir un retraso en la identificaci&#243;n de nuevos casos de neoplasia<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">17</span></a>&#46; En el caso de los tumores hematol&#243;gicos&#44; en los que los pacientes suelen mostrar niveles m&#225;s altos de inmunosupresi&#243;n y mayor susceptibilidad a infecciones&#44; la COVID-19 pudiera tener un impacto distinto respecto a pacientes que comenzaran con tumores s&#243;lidos<a class="elsevierStyleCrossRef" href="#bib0240"><span class="elsevierStyleSup">18</span></a>&#46; De hecho&#44; en un estudio en el que se analizaron pacientes hospitalizados con c&#225;ncer seg&#250;n el tipo de neoplasia se observ&#243; que aquellos con neoplasias hematol&#243;gicas mostraron mayores tasas de infecci&#243;n por SARS-COV-2 y requirieron m&#225;s intervenciones de soporte que aquellos con otro tipo de tumor&#46; Adem&#225;s&#44; comparando con pacientes oncol&#243;gicos sin COVID-19&#44; la leucemia fue el &#250;nico tipo de c&#225;ncer que registr&#243; una mayor mortalidad durante el per&#237;odo de pandemia analizado<a class="elsevierStyleCrossRef" href="#bib0245"><span class="elsevierStyleSup">19</span></a>&#46;</p><p id="par0125" class="elsevierStylePara elsevierViewall">Las estimaciones para 2021 muestran que los linfomas difusos de c&#233;lulas B grandes se convierten en el subtipo m&#225;s frecuente entre las neoplasias linfoides a la par que el mieloma m&#250;ltiple&#46; Los linfomas difusos de c&#233;lulas B grandes son por s&#237; mismos un grupo heterog&#233;neo de entidades biol&#243;gicamente distintas&#44; que pueden ser agresivas pero curables y que se traducen en una proliferaci&#243;n clonal de c&#233;lulas B germinales o posgerminales malignas&#46; A pesar de las numerosas investigaciones&#44; el tratamiento est&#225;ndar para la gran mayor&#237;a de este tipo de neoplasias es&#44; desde hace d&#233;cadas&#44; la quimioterapia R-CHOP &#40;rituximab&#44; ciclofosfamida&#44; doxorrubicina&#44; vincristina y prednisona&#41; pero el control de la enfermedad disminuye de un 90&#37; a un 60&#37; de los casos cuando se diagnostica en estados avanzados<a class="elsevierStyleCrossRef" href="#bib0250"><span class="elsevierStyleSup">20</span></a>&#59; en este sentido&#44; es necesario intensificar los esfuerzos para evitar un retraso diagn&#243;stico excesivo que implique identificar casos en estadios m&#225;s avanzados&#46; Por su parte&#44; el mieloma m&#250;ltiple se asocia con disfunci&#243;n inmunitaria tanto celular como humoral y causa un estado de inmunosupresi&#243;n generalizada&#44; dejando a los pacientes especialmente vulnerables a las infecciones&#46; Debido al envejecimiento de la poblaci&#243;n&#44; tiene sentido que la incidencia del mieloma m&#250;ltiple pueda aumentar considerablemente en los pr&#243;ximos a&#241;os dado que se diagnostica especialmente en mayores de 65 a&#241;os&#46; Esta enfermedad supone importantes recursos humanos y sanitarios en el seguimiento y tratamiento de los pacientes afectados<a class="elsevierStyleCrossRefs" href="#bib0255"><span class="elsevierStyleSup">21&#44;22</span></a>&#46; Adem&#225;s&#44; en un porcentaje considerable de los pacientes geri&#225;tricos con mieloma m&#250;ltiple&#44; las indicaciones terap&#233;uticas no est&#225;n claras puesto que los beneficios de las opciones terap&#233;uticas se han evaluado en ensayos cl&#237;nicos en los que los participantes suelen ser m&#225;s j&#243;venes y con menos comorbilidades&#46; Por otro lado&#44; el efecto de COVID-19 u otras posibles nuevas infecciones en pacientes con mieloma m&#250;ltiple es particularmente preocupante debido a una inmunosupresi&#243;n asociada con la enfermedad que no est&#225; bien esclarecida&#46;</p><p id="par0130" class="elsevierStylePara elsevierViewall">Con relaci&#243;n a las proyecciones de las neoplasias mieloides&#44; los datos muestran que la trombocitemia esencial sigue siendo el subtipo m&#225;s incidente&#46; Los pacientes diagnosticados con esta enfermedad&#44; en general&#44; cuentan con un buen pron&#243;stico especialmente si no evoluciona a policitemia vera o mielofibrosis primaria<a class="elsevierStyleCrossRefs" href="#bib0265"><span class="elsevierStyleSup">23&#44;24</span></a>&#46; La estratificaci&#243;n del riesgo en base a los antecedentes de trombosis&#44; mutaciones JAK2&#44; edad &#40;&#62;<span class="elsevierStyleHsp" style=""></span>60 a&#241;os&#41; y factores de riesgo cardiovascular pueden influir en la estrategia terap&#233;utica y el pron&#243;stico<a class="elsevierStyleCrossRef" href="#bib0270"><span class="elsevierStyleSup">24</span></a>&#46; Algunos estudios apuntan a que una parte de los diagn&#243;sticos de trombocitemia esencial a&#250;n se realizan despu&#233;s de un evento tromb&#243;tico&#44; y que se someten a la primera evaluaci&#243;n hematol&#243;gica despu&#233;s de una larga historia de trombocitosis<a class="elsevierStyleCrossRef" href="#bib0275"><span class="elsevierStyleSup">25</span></a>&#46; En este sentido&#44; ser&#237;a recomendable incidir en los criterios diagn&#243;sticos entre los m&#233;dicos de atenci&#243;n primaria&#44; intensificando la atenci&#243;n en aquellos pacientes con historia de trombocitosis&#46;</p><p id="par0135" class="elsevierStylePara elsevierViewall">El grupo de los SMD y mieloproliferativos&#44; sobre todo la leucemia mielomonoc&#237;tica cr&#243;nica pero tambi&#233;n&#44; aunque son menos prevalentes&#44; la leucemia mieloide cr&#243;nica at&#237;pica y los SMD&#47;mieloproliferativos inclasificables&#44; aumentan en 2021 respecto a per&#237;odos previos&#46; Es especialmente llamativo el aumento de los SMD con displasia multilinaje&#46; La etiolog&#237;a de esta enfermedad <span class="elsevierStyleItalic">de novo</span> es desconocida&#44; y la exposici&#243;n a determinados t&#243;xicos ambientales podr&#237;an incrementar el riesgo<a class="elsevierStyleCrossRefs" href="#bib0165"><span class="elsevierStyleSup">3&#44;26</span></a>&#46; Se estima que un 15&#37; y un 28&#37; evolucionaran a leucemia aguda a los 2 y 5 a&#241;os del diagn&#243;stico&#44; respectivamente<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">3</span></a>&#46; Entre un 30&#37; y un 50&#37; de los SMD presentan alteraciones citogen&#233;ticas y hasta en un 90&#37; se identifican mutaciones cuando se emplea un panel dirigido<a class="elsevierStyleCrossRef" href="#bib0285"><span class="elsevierStyleSup">27</span></a>&#59; dichas alteraciones marcar&#225;n el pron&#243;stico de los pacientes&#44; por lo que la detecci&#243;n de estas alteraciones debe ser un objetivo prioritario en el &#225;mbito asistencial&#46; De hecho&#44; las t&#233;cnicas de citogen&#233;tica y secuenciaci&#243;n han revolucionado el diagn&#243;stico en los &#250;ltimos a&#241;os y puede ser&#44; en parte&#44; el motivo del aumento de la incidencia de los SMD en detrimento de la mayor&#237;a de las neoplasias inespec&#237;ficas&#46; Adem&#225;s&#44; ayudan a identificar respondedores a tratamiento y en consecuencia se establecen mejoras en la supervivencia&#44; as&#237; como en la eficacia de la utilizaci&#243;n de f&#225;rmacos y los costes de los tratamientos<a class="elsevierStyleCrossRefs" href="#bib0290"><span class="elsevierStyleSup">28&#44;29</span></a>&#46;</p><p id="par0140" class="elsevierStylePara elsevierViewall">Un dato interesante que se deriva de este estudio es la disminuci&#243;n de neoplasias hematol&#243;gicas inespec&#237;ficas&#46; Tal y como se ha comentado anteriormente&#44; los continuos avances en las t&#233;cnicas diagn&#243;sticas gen&#233;ticas y moleculares&#44; as&#237; como la formaci&#243;n espec&#237;fica que se ha ido realizando a lo largo de los &#250;ltimos a&#241;os en los registros de c&#225;ncer poblacional han repercutido en una optimizaci&#243;n en el diagn&#243;stico y codificaci&#243;n de este tipo de enfermedad&#46; No obstante&#44; este hallazgo debe tomarse con prudencia dado que el conjunto de casos inespec&#237;ficos no forman individualmente un tipo de enfermedad&#44; y por tanto&#44; no constituyen una variable aleatoria en la implementaci&#243;n del modelo estad&#237;stico&#46;</p><p id="par0145" class="elsevierStylePara elsevierViewall">Otra de las limitaciones de este estudio podr&#237;a estar relacionada con sesgos de codificaci&#243;n en los subgrupos espec&#237;ficos de neoplasia entre los distintos registros poblacionales&#59; sin embargo&#44; globalmente&#44; los datos de los grandes grupos de enfermedades son concordantes y&#44; adem&#225;s&#44; se ha observado una mejora significativa en la codificaci&#243;n en cuanto a los casos inespec&#237;ficos en los &#250;ltimos a&#241;os y en todos los registros participantes en este estudio<a class="elsevierStyleCrossRef" href="#bib0300"><span class="elsevierStyleSup">30</span></a>&#46; Por otro lado&#44; es necesario se&#241;alar la baja potencia estad&#237;stica para algunos subtipos de neoplasia muy poco frecuentes que pudiera haber influido en las proyecciones&#46;</p><p id="par0150" class="elsevierStylePara elsevierViewall">Las proyecciones en el n&#250;mero y las tasas de nuevos casos de c&#225;ncer deben ser interpretadas con cautela dado que est&#225;n basadas en subpoblaciones que est&#225;n variando considerablemente en los &#250;ltimos a&#241;os debido a los refinamientos en el diagn&#243;stico y codificaci&#243;n&#46; Sin embargo&#44; los m&#233;todos y la estrategia utilizados en el an&#225;lisis de los datos proporcionan una estimaci&#243;n razonablemente precisa de la incidencia actual de las neoplasias hematol&#243;gicas en Espa&#241;a&#44; en base a la mayor cobertura poblacional que proporcionan actualmente los registros en este pa&#237;s&#46; Finalmente&#44; las proyecciones aqu&#237; presentadas pudieran estar subestimadas debido a la mejora continua en cuanto a la identificaci&#243;n de neoplasias hematol&#243;gicas por parte de los registros poblacionales y a la aparici&#243;n de herramientas diagn&#243;sticas m&#225;s eficaces en t&#233;rminos moleculares&#46; En todo caso&#44; la mejora en todos estos aspectos refuerza la validez de los datos poblacionales y fortalece la toma de decisiones de planificaci&#243;n sanitaria que se pueden establecer en base a los mismos&#46;</p><p id="par0155" class="elsevierStylePara elsevierViewall">En conclusi&#243;n&#44; conocer el n&#250;mero de casos esperados de neoplasias hematol&#243;gicas para 2021 puede ser de utilidad para valorar el impacto de la infecci&#243;n de SARS-COV-2 en el diagn&#243;stico de este tipo de enfermedad cuando se disponga de informaci&#243;n real en cuanto a la incidencia del c&#225;ncer&#46; Adem&#225;s&#44; las proyecciones aqu&#237; presentadas pueden servir de base para la planificaci&#243;n de recursos dirigidos a implementar m&#233;todos diagn&#243;sticos punteros como la citogen&#233;tica y la secuenciaci&#243;n de ADN de nueva generaci&#243;n&#44; que informan no solo del diagn&#243;stico&#44; sino tambi&#233;n del pron&#243;stico&#46; Asimismo&#44; pueden servir de orientaci&#243;n para la previsi&#243;n de tratamientos dirigidos&#44; algunos de los cuales revierten en mayores tasas de supervivencia&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicto de intereses</span><p id="par0160" class="elsevierStylePara elsevierViewall">No existe conflicto de intereses&#46;</p></span></span>"
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                  \t\t\t\t">1&#46;1 Linfoma de Hodgkin cl&#225;sico&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">1&#46;2 Linfoma de Hodgkin&#44; predom&#46; linfoc&#237;tico nodular&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">2&#46;1 Neoplasias linfoides de c&#233;lulas precursoras&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">2&#46;1&#46;1 Leucemia&#47;linfoma linfobl&#225;stica B&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">2&#46;1&#46;2 Leucemia&#47;linfoma linfobl&#225;stica T&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">2&#46;1&#46;3 Leucemia&#47;linfoma linfobl&#225;stica&#44; SAI&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">2&#46;2&#41; Neoplasias de c&#233;lulas B maduras&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">2&#46;2&#46;1 Leucemia linfoc&#237;tica cr&#243;nica &#47;Linfoma linfoc&#237;tico de c&#233;lulas peque&#241;as&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">1&#46;281 &#40;1&#46;176-1&#46;385&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">828 &#40;745-911&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">3&#44;43 &#40;3&#44;08-3&#44;77&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">3&#44;09 &#40;2&#44;78-3&#44;4&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">2&#46;2&#46;2 Leucemia prolinfoc&#237;tica&#44; c&#233;lulas B&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t">7 &#40;0-17&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">10 &#40;4-16&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">2&#46;2&#46;3 Leucemia de c&#233;lulas pilosas&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
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                  \t\t\t\t">2&#46;2&#46;4 Linfoma marginal&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">657 &#40;569-745&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">2&#44;33 &#40;1&#44;98-2&#44;68&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">2&#46;2&#46;5 Linfoma linfoplasmac&#237;tico &#47; macroglobulinemia de Waldenstr&#246;m&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">0&#44;61 &#40;0&#44;44-0&#44;77&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2&#46;2&#46;6 Mieloma m&#250;ltiple&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t">1&#46;914 &#40;1&#46;763-2&#46;065&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">8&#44;25 &#40;7&#44;6-8&#44;9&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">1&#46;434 &#40;1&#46;312-1&#46;555&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">5&#44;94 &#40;5&#44;43-6&#44;44&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">5&#44;35 &#40;4&#44;91-5&#44;78&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2&#46;2&#46;7 Linfoma folicular&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">1&#46;279 &#40;1&#46;176-1&#46;382&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">5&#44;51 &#40;5&#44;07-5&#44;96&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">5&#44;63 &#40;5&#44;18-6&#44;08&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">1&#46;328 &#40;1&#46;218-1&#46;438&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">5&#44;5 &#40;5&#44;04-5&#44;96&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t">4&#44;95 &#40;4&#44;52-5&#44;38&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2&#46;2&#46;8 Linfoma de c&#233;lulas del manto&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t">234 &#40;189-280&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">1&#44;01 &#40;0&#44;81-1&#44;21&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t">1&#44;03 &#40;0&#44;82-1&#44;24&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">112 &#40;79-145&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">0&#44;46 &#40;0&#44;33-0&#44;6&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t">0&#44;42 &#40;0&#44;29-0&#44;54&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2&#46;2&#46;9 Linfoma difuso de c&#233;lulas B grandes&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t">1&#46;804 &#40;1&#46;662-1&#46;945&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
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