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目的 :了解唐氏综合征(Down’s syndrome,DS)患儿发生血液系统疾病的临床特点,提高DS患儿合并血液病的诊断与治疗水平。方法:回顾性分析4例DS合并恶性血液病患儿资料并复习文献,了解DS患儿合并恶性血液病的临床特点及治疗反应。结果:4例合并血液病的DS患儿中,1例为急性淋巴细胞白血病,1例为急性巨核细胞白血病,2例为巨核细胞系的病态造血。急性淋巴细胞白血病患儿表现出了对甲氨蝶呤的明显毒性,而急性巨核细胞白血病显示了对化疗的良好反应。结论:DS患儿特殊基因背景决定了其生后特征性血液病表现及治疗反应。
Objective: To understand the clinical features of blood diseases in children with Down’s syndrome (DS) and to improve the diagnosis and treatment of blood diseases in children with DS. Methods: A retrospective analysis of 4 cases of DS with hematologic malignancies in children and review the literature to understand the clinical characteristics of DS children with hematologic malignancies and response to treatment. Results: Of 4 DS patients with blood diseases, 1 was acute lymphoblastic leukemia, 1 was acute megakaryocyte leukemia and 2 was pathological hematopoiesis of megakaryocyte. Children with acute lymphoblastic leukemia show significant toxicity to methotrexate, whereas acute megakaryoblastic leukemia shows good response to chemotherapy. Conclusion: The special genetic background of DS children determines their postnatal characteristic hematologic manifestations and response to treatment.