Neoplasias mieloproliferativas crónicas. Clasificación. Leucemia mieloide crónica

J.M. Aspa-Cilleruelo , G. López de Hontanar Torres , M. Menor Gómez , E. Magro Mazo
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引用次数: 0

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.
慢性骨髓增生性肿瘤。分类。慢性骨髓性白血病
骨髓增殖性肿瘤(MPN)是起源于造血祖细胞的增殖性克隆疾病。它们包括不同的疾病,其中一种或多种细胞系增殖可能相互重叠。诊断必须综合考虑分析、临床、分子和组织学数据。它们都是慢性疾病。2022 年世卫组织公布新分类后,这些疾病包括慢性髓性白血病(CML)、真性多血细胞增多症、原发性血小板增多症、原发性骨髓纤维化、慢性嗜中性粒细胞白血病、慢性嗜酸性粒细胞白血病和未分类的多发性骨髓瘤。临床表现继发于细胞增殖,包括体征、贫血、感染、出血和血栓事件,以及与脾脏肿大有关的表现(在所有这些实体中都很常见)。除使用酪氨酸激酶抑制剂(TKI)治疗的 CML 外,其余多发性骨髓瘤的治疗主要包括控制疾病引起的症状(瘙痒、头痛、贫血、脾肿大等)和细胞再生治疗。
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