儿童慢性嗜酸粒细胞白血病1例并文献复习

Childhood chronic eosinophilic leukemia:a case report and review of literatures

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 elucidate the clinical features, cytogenetics, diagnosis, treatment and prognosis of chronic eosinophilic leukemia（ CEL）, we analyzed a child with CEL who has extremely elevated eosinophils and reviewed associated literatures. Methods Clinical case analysis and articles review were used. Results Anemia, hemorrhage, splenohepatomagly, lymphadenectasis and systemic toxicity caused by released cytokines are major clinical features. Platelet derived growth factor receptor （PDGFR） gene is always involved in the chromosomal translocations and Wilms＇ tumor gene is always up - regulated in CEL, which is the main difference from idopathic hypereosinophilic syndrome （IHES）. Except for chemotherapy, immunotherapy with IFN - α, targeted therapy with protein tyrosine kinases inhibator and stem cell transplantation （SCT） are also useful. Prognosis of CEL is poor. Conclusions Chronic eosinophilic leukemia （CEL） is a rare kind of chronic myeloprolifierative disorders, especially in childhood. Except for the common symptoms of leukemia, system damage caused by cytokines should be paid more attention. Diagnosis depended on cytogenetics. The effects of several treatments were not certain, among which SCT was the best till now. White cell detachment and plasmapheresis are important to prevent the systemic damage of released cytokines.