Vol. 14. Núm. 20.
Páginas 1149-1156 (Noviembre 2024)

Actualización
Neoplasias mieloproliferativas crónicas. Clasificación. Leucemia mieloide crónica

Chronic myeloproliferative neoplasms. Classification. Chronic myeloid leukemia

J.M. Aspa-CillerueloaG. López de Hontanar TorresbM. Menor GómezbE. Magro Mazob
Doi : https://10.1016/j.med.2024.10.019
Opciones
Resumen

Las neoplasias mieloproliferativas (NMP) son trastornos clonales proliferativos que se originan a nivel de los progenitores hematopoyéticos, comprenden diferentes enfermedades que pueden llegar a solaparse donde proliferan una o más líneas celulares, y cuyo diagnóstico debe ser integrado, teniendo en cuenta los datos analíticos, clínicos, moleculares e histológicos. Son enfermedades de curso crónico. Tras publicar la OMS en 2022 la nueva clasificación, dichas entidades incluyen: leucemia mieloide crónica (LMC), policitemia vera, trombocitemia esencial, mielofibrosis primaria, leucemia neutrofílica crónica, leucemia eosinofílica crónica y NMP no clasificadas. Las manifestaciones clínicas son secundarias a la proliferación celular y son: los síntomas constitucionales, el síndrome anémico, las infecciones, los eventos hemorrágicos y trombóticos y las manifestaciones relacionadas con la esplenomegalia (que es frecuente en todas estas entidades). Con excepción de la LMC que se trata con inhibidores de la tirosina-cinasa (ITK), en el resto de las NMP el tratamiento consiste fundamentalmente en el control de los síntomas derivados de la enfermedad (prurito, cefalea, anemia, esplenomegalia, etc.) y el tratamiento con citorreductores.

Palabras clave
Neoplasia mieloproliferativa crónica
Leucemia mieloide crónica
Trombocitemia esencial
Policitemia vera
Leucemia eosinofílica crónica
Leucemia neutrofílica crónica
Abstract

Myeloproliferative neoplasms (MPN) are proliferative clonal disorders that originate in hematopoietic progenitor cells. They include different diseases that may overlap in which one or more cell lines proliferate. The diagnosis must be integrated, taking into account analytical, clinical, molecular, and histological data. They are chronic diseases. Following publication of the new WHO classification in 2022, these diseases include chronic myeloid leukemia (CML), polycythemia vera, essential thrombocythemia, primary myelofibrosis, chronic neutrophilic leukemia, chronic eosinophilic leukemia, and unclassified MPNs. Clinical manifestations are secondary to cell proliferation and include constitutional symptoms, anemia, infections, hemorrhagic and thrombotic events, and manifestations related to splenomegaly (which is common in all these entities). With the exception of CML, which is treated with tyrosine kinase inhibitors (TKI), treatment for the rest of MPNs mainly consists of controlling the symptoms arising from the disease (pruritus, headache, anemia, splenomegaly, etc.) and cytoreductive treatment.

Keywords
Chronic myeloproliferative neoplasms
Chronic myeloid leukemia
Essential thrombocythemia
Polycythemia vera
Chronic eosinophilic leukemia
Chronic neutrophilic leukemia

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