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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Pre-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 understanding of Pre-ALL in children with acute lymphoblastic leukemia. Methods Six children with Pre-ALL admitted to Xi’an Children’s Hospital from 1997 to 2004 were analyzed for clinical experience and laboratory characteristics, and relevant literature was reviewed. Results 4 males and 2 females, aged 2 months and 3 months to 8 years and 7 months, 6 cases were fever, anemia, bleeding. Newly diagnosed as aplastic anemia (AA) in 5 cases, bone marrow dysplasia syndrome (MDS) in 1 case. Four cases had liver, spleen and lymph node enlargement (non-simultaneous), and the number of three-line cells decreased. Bone marrow aspirate smears showed that the hyperplasia was reduced in 4 cases and the severe degeneration in 2 cases. The bone marrow biopsy showed that the adipose tissue increased in 4 cases and the hematopoietic tissue Decreased; 2 cases of dry pumping accompanied by fibrous tissue hyperplasia, 2 cases of pathological hematopoiesis. With or without glucocorticoid treatment, in 1 to 9 months were converted to acute lymphoblastic leukemia (ALL). 3 cases received VDLP regimen was completely relieved. Conclusions Six cases turned into ALL, which were sensitive to chemotherapy and some of them had morphological abnormalities.