Diamantopoulos Panagiotis T, Viniou Nora-Athina
First Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, Athens, Greece.
Front Oncol. 2021 Nov 17;11:722507. doi: 10.3389/fonc.2021.722507. eCollection 2021.
Atypical chronic myelogenous leukemia (aCML), negative is a rare myelodysplastic/myeloproliferative neoplasm, usually manifested with hyperleukocytosis without monocytosis or basophilia, organomegaly, and marked dysgranulopoiesis. In this review, we will discuss the classification and diagnostic criteria of aCML, as these have been formulated during the past 30 years, with a focus on the recent advances in the molecular characterization of the disease. Although this entity does not have a definitive molecular profile, its molecular characterization has contributed to a better understanding and more accurate classification and diagnosis of aCML. At the same time, it has facilitated the identification of adverse prognostic factors and the stratification of patients according to their risk for leukemic transformation. What is more, the molecular characterization of the disease has expanded our therapeutic choices, thoroughly presented and analyzed in this review article.
非典型慢性粒细胞白血病（aCML），阴性是一种罕见的骨髓增生异常/骨髓增殖性肿瘤，通常表现为白细胞增多而无单核细胞增多或嗜碱性粒细胞增多、器官肿大以及明显的粒细胞生成异常。在本综述中，我们将讨论aCML的分类和诊断标准，这些标准是在过去30年中制定的，重点是该疾病分子特征的最新进展。尽管该实体没有明确的分子特征，但其分子特征有助于更好地理解aCML，并进行更准确的分类和诊断。同时，它有助于识别不良预后因素，并根据患者白血病转化的风险进行分层。此外，该疾病的分子特征扩大了我们的治疗选择，本综述文章将对此进行全面介绍和分析。
