Lynott ANNE, Ravandi-Kashani FARHAD, Giles FRANCIS J.
Department of Leukemia, University of Texas MD Anderson Cancer Center, Houston, Texas.
Hematology. 2000;4(6):487-493.
Conditions associated with increased peripheral blood and bone marrow eosinophil count may be reactive, clonal or idiopathic. Clonal eosinophilic disorders are characterized by increased production of eosinophils alongside a clone of malignant cells. In these patients, the eosinophils can either be demonstrated as being part of the malignant clone or produced as a result of cytokine production by the malignant clone. Criteria for the diagnosis of idiopathic hypereosinophilic syndrome (HES) include the exclusion of other known causes of hypereosinophilia. A few patients with the initial diagnosis of HES develop clonal disorders manifested by granulocytic sarcoma or acute leukemia. We report a patient with a nine year history of HES before progressing to chloroma and acute leukemia. Cytogenetic studies on the bone marrow specimen revealed trisomy 8. This report and others in the literature support the concept that at least some cases of HES are as yet unidentified clonal diseases. Cytogenetic studies are therefore recommended at diagnosis and during the follow up of patients with HES.
外周血和骨髓嗜酸性粒细胞计数增加相关的情况可能是反应性、克隆性或特发性的。克隆性嗜酸性粒细胞疾病的特征是嗜酸性粒细胞生成增加以及出现恶性细胞克隆。在这些患者中,嗜酸性粒细胞既可以表现为恶性克隆的一部分,也可以是由恶性克隆产生的细胞因子所致。特发性高嗜酸性粒细胞综合征(HES)的诊断标准包括排除其他已知的嗜酸性粒细胞增多原因。一些最初诊断为HES的患者会发展为以粒细胞肉瘤或急性白血病为表现的克隆性疾病。我们报告1例有9年HES病史,之后进展为绿色瘤和急性白血病的患者。对骨髓标本的细胞遗传学研究显示8号染色体三体。本报告及文献中的其他报告支持这样一种观点,即至少部分HES病例是尚未明确的克隆性疾病。因此,建议在HES患者诊断时及随访期间进行细胞遗传学研究。