小儿急性淋巴细胞白血病前期6例分析及文献复习

Analysis of 6 children with the preceding acute lymphoblastic leukemia and review of literature

目的提高对小儿急性淋巴细胞白血病前期（Pre-ALL）的认识。方法对西安市儿童医院1997-2004收治的6例Pie-ALL患儿的临床经过及实验室的特点进行分析，并复习相关文献。结果男4例，女2例，年龄2岁3个月至8岁7个月，6例均有发热、贫血、出血。初诊为再生障碍性贫血（AA）5例、骨髓异常增生综合征（MDS）1例。4例出现肝、脾、淋巴结肿大（非同时），三系细胞减少，骨髓穿刺涂片示：增生减低4例，重度减低2例；骨髓病理活检：4例均示脂肪组织增多，造血组织减少；2例出现干抽的患儿伴有纤维组织增生，2例出现病态造血。经或不经糖皮质激素治疗，在1～9个月内均转变为急性淋巴细胞白血病（ALL）。3例接受VDLP方案治疗获完全缓解。结论6例转变为ALL，对化疗敏感，部分Pre-ALL有形态学异常。

Objective To improve the diagnosis of preceding acute lymphoblastic leukemia （Pre-ALL）. Methods Clinical and laboratory characteristics were reviewed in 6 cases of Pre-ALL admitted between 1997 and 2004. Results Four males and 2 females, aged from 2 to 8 years old, all the cases had fever,anemia and bleeding, four cases with hepatosplenomegaly, enlargement of lymphonode and pancytopenia, four cases with hypoplasia of bone marrow; more fat tissue and less hematopoietic tissue were found in the four cases of bone marrow biopsy;two cases with dry-draw of bone marrow had proliferation of fibrotic tissue ; two children pccurred pathologic hematopoiesis. All these patients transformed to ALL within one to nine months with or without steroids therapy. Three of the ALL were given VDLP and got complete relief. Conclusion Some patients diagnosed as aplastic anemia at first can transform to ALL,with good response to chemotherapy. Part of the children with pre-ALL may show abnormal morphology in bone marrow.