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目的通过分析一例嗜酸细胞异常增高的儿童慢性嗜酸粒细胞白血病,结合文献阐明该病的临床特点、细胞遗传学改变、诊断、治疗及预后。方法临床病例分析及文献综述。结果该病人及文献复习显示本病临床特点主要为贫血、出血、肝脾淋巴结肿大以及嗜酸细胞破坏后释放出大量细胞因子引起全身各脏器损害的相关症状;细胞遗传学改变通常为涉及血小板衍生的生长因子受体基因的染色体异位及Wilms瘤基因的高表达;与特发性嗜酸细胞增生症的鉴别主要为细胞遗传学的改变,前者多有克隆性染色体异常以及癌基因的的异常表达;治疗除化疗外还有IFN-α的免疫治疗,酪氨酸激酶抑制剂靶向治疗以及干细胞移植;该病预后不佳。结论:该病在儿童非常罕见,除白血病常见症状外应重视大量细胞因子引起的各脏器相关症状,确诊主要靠细胞遗传学,治疗方法虽多但疗效多不确切,干细胞移植是目前较为理想的方法,在治疗过程中辅以白细胞分离和血浆置换对于防止嗜酸细胞破坏后大量细胞因子对各脏器的损害十分必要。
Objective To analyze the clinical features, cytogenetic changes, diagnosis, treatment and prognosis of children with chronic eosinophocytic leukemia by analyzing an eosinophilic abnormality in children. Methods Clinical cases and literature review. Results The patient and literature review showed that the main clinical features of the disease were anemia, hemorrhage, hepatosplenomegaly and eosinophil disruption, which released a large number of cytokines and related symptoms of damage to the organs of the whole body. Cytogenetic changes usually involved Platelet-derived growth factor receptor gene chromosomal abnormalities and Wilms tumor gene expression; and idiopathic eosinophilia identified mainly cytogenetic changes, the former mostly clonal chromosomal abnormalities and oncogenes ; In addition to chemotherapy there are immunotherapy IFN-α, tyrosine kinase inhibitor targeted therapy and stem cell transplantation; poor prognosis of the disease. Conclusion: The disease is very rare in children, in addition to the common symptoms of leukemia should pay attention to a large number of cytokines caused by the symptoms associated with various organs, the diagnosis depends mainly on cytogenetics, although the treatment of many and more curative effect, stem cell transplantation is more ideal Of the method, supplemented by leukocyte isolation and plasma exchange in the course of treatment is essential for preventing the damage of a large number of cytokines to various organs after the destruction of eosinophils.