PDF(Pubmed)
Abstract：
BACKGROUND: Atypical chronic myeloid leukemia (aCML) is a highly aggressive type of blood cancer that falls under the category of myelodysplastic/myeloproliferative neoplasms (MDS/MPN). In the fifth edition of the WHO classification of tumors, this category has been renamed MDS/MPN with neutrophilia. Although eosinophilia is commonly observed in blood cancers, it is rarely seen in aCML.
METHODS: This study presents a case of aCML that was diagnosed six years after the patient developed eosinophilia. The patient had undergone tests to rule out other primary and secondary diseases, but the eosinophilia remained unexplained. Treatment with corticosteroids and hydroxyurea had proven ineffective. Six years later, the patient experienced an increase in white blood cells, primarily neutrophils. After ruling out other possible diagnoses, a combination of morphologic and molecular genetic findings led to the diagnosis of aCML. The patient responded well to treatment with azacitidine.
CONCLUSIONS: This study summarizes the current state of aCML diagnosis and management and discusses the possible connection between eosinophilia and aCML.
METHODS: This study presents a case of aCML that was diagnosed six years after the patient developed eosinophilia. The patient had undergone tests to rule out other primary and secondary diseases, but the eosinophilia remained unexplained. Treatment with corticosteroids and hydroxyurea had proven ineffective. Six years later, the patient experienced an increase in white blood cells, primarily neutrophils. After ruling out other possible diagnoses, a combination of morphologic and molecular genetic findings led to the diagnosis of aCML. The patient responded well to treatment with azacitidine.
CONCLUSIONS: This study summarizes the current state of aCML diagnosis and management and discusses the possible connection between eosinophilia and aCML.
摘要：
背景：非典型慢性粒细胞白血病（aCML）是一种高度侵袭性类型的血癌，属于骨髓增生异常/骨髓增殖性肿瘤（MDS/MPN）。在世界卫生组织第五版的肿瘤分类中，该类别已更名为中性粒细胞增多症的MDS/MPN。尽管嗜酸性粒细胞增多通常在血癌中观察到，在aCML中很少见。
方法：本研究提供一例aCML病例，该病例在患者出现嗜酸性粒细胞增多6年后确诊。病人接受了检查以排除其他原发性和继发性疾病，但嗜酸性粒细胞增多仍无法解释.用皮质类固醇和羟基脲治疗已证明无效。六年后,病人出现了白细胞的增加,主要是中性粒细胞。排除了其他可能的诊断后，形态学和分子遗传学的结合发现导致了aCML的诊断.患者对阿扎胞苷治疗反应良好。
结论：本研究总结了aCML的诊断和治疗现状，并讨论了嗜酸性粒细胞增多和aCML之间的可能联系。
方法：本研究提供一例aCML病例，该病例在患者出现嗜酸性粒细胞增多6年后确诊。病人接受了检查以排除其他原发性和继发性疾病，但嗜酸性粒细胞增多仍无法解释.用皮质类固醇和羟基脲治疗已证明无效。六年后,病人出现了白细胞的增加,主要是中性粒细胞。排除了其他可能的诊断后，形态学和分子遗传学的结合发现导致了aCML的诊断.患者对阿扎胞苷治疗反应良好。
结论：本研究总结了aCML的诊断和治疗现状，并讨论了嗜酸性粒细胞增多和aCML之间的可能联系。
