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目的探讨急性淋巴细胞白血病患者长期化疗后发生治疗相关骨髓增生异常综合征白血病(t-MDS/AML)的临床特征及预后。方法观察一例儿童急性淋巴细胞白血病经化疗后演变为幼年慢性粒-单核细胞白血病(JMML)的临床演变过程及治疗结果并进行相关文献复习。结果1例急性淋巴细胞白血病患儿经3年正规化疗停药后3月,外周血白细胞进行性增高并出现原始粒、单核细胞。各项检查支持幼年慢性粒-单核细胞白血病诊断。根据2000年WHO造血组织和淋巴组织肿瘤分类方法,归于MDS/MPD一类疾病。患者迅速进展为AML-M4b,治疗无效死亡。结论t-MDS比原发性MDS病情进展更快,预后更差。t-AML临床预后不良,骨髓移植及大剂量化疗仅对部分患者有效。应进一步加强儿童ALL患者的合理用药,根据不同危险度分型,选取相应治疗强度化疗方案,以减少t-MDS/AML发生的可能。
Objective To investigate the clinical features and prognosis of patients with acute myeloid leukemia (T-MDS / AML) treated with long-term chemotherapy after treatment of myelodysplastic syndrome leukemia (AML). Methods A case of children with acute lymphoblastic leukemia after chemotherapy evolved into juvenile chronic myelomonocytic leukemia (JMML) clinical evolution and treatment of the results and literature review. Results One case of acute lymphoblastic leukemia in children after 3 years of formal chemotherapy withdrawal in March, the peripheral leukocytes progressive increase and primary particles, monocytes. Various tests support the diagnosis of juvenile chronic granulocytic-monocytic leukemia. According to 2000 WHO hematopoietic tissue and lymphoid tumor classification method, attributed to MDS / MPD a class of diseases. The patient progressed rapidly to AML-M4b and died of ineffective treatment. Conclusion t-MDS progresses faster than primary MDS and its prognosis is worse. T-AML poor clinical prognosis, bone marrow transplantation and high-dose chemotherapy is only effective in some patients. Should be further strengthened the rational use of drugs in children with ALL, according to the different risk classification, select the appropriate treatment intensity of chemotherapy to reduce the possibility of t-MDS / AML.