幼年型粒单核细胞白血病的临床及实验室特征分析

Clinical and laboratory characteristics of juvenile myelomonocytic leukemia

目的分析幼年型粒单核细胞白血病(JMML)的临床与实验室特征。方法对10例初诊JMML患者的临床特征及实验室结果进行回顾性分析,并与同期确诊的28例骨髓增生异常综合征(MDS)、44例慢性粒细胞白血病(CML)患儿进行对比。结果与CML及MDS患者相比,JMML患儿皮疹、瘀斑及淋巴结肿大的出现几率较高,而血清胆碱酯酶(ChE)最低。JMML患儿抗碱血红蛋白(HbF)最高,白细胞计数高于MDS组而低于CML组,粒红比与病态造血比例分别低于CML与MDS组;JMML组成熟单核细胞标记CD14表达较高,髓系标记CD33、CD11b、CD13及CD15的表达高于MDS组而低于CML组,差异均有统计学意义(P<0.05),CD7及CD2高于CML组而低于MDS组(P<0.05)。结论 JMML患儿皮疹、瘀斑、淋巴结肿大以及ChE降低较为多见,骨髓病态造血现象较少,CD14表达明显增高。

Objective To study the clinical and laboratory characteristics of juvenile myelomonocytic leukemia（JMML）. Methods The clinical characteristics and laboratory results were retrospectively analyzed in 10 children with newly diagnosed JMML. They were compared with those of 28 children with myelodysplastic syndrome（MDS） and 44 children with chronic myeloid leukemia（CML）. Results Compared with the children with CML or MDS, the children with JMML had significantly higher rates of skin rashes, ecchymosis, and lymphadenectasis, a significantly lower serum cholinesterase（Ch E） level, and a significantly higher fetal hemoglobin level（P〈0.05）. The white blood cell count of children with JMML was significantly higher than that of children with MDS, but significantly lower than that of children with CML（P〈0.05）. In addition, the myeloid/erythroid ratio and rate of dyshaematopoiesis were significantly lower in children with JMML than those in children with CML or MDS. The children with JMML had a significantly higher expression of mature monocyte marker CD14 than those with CML or MDS（P〈0.05）. The levels of myeloid markers CD33, CD11 b, CD13, and CD15 in children with JMML were significantly higher than those in children with MDS, but significantly lower than those in children with CML（P〈0.05）. The levels of CD2 and CD7 in children with JMML were higher than those in children with CML, but lower than those in children with MDS（P〈0.05）. Conclusions Skin rashes, ecchymosis, lymphadenectasis, and ChE reduction are more common in children with JMML than in those with CML or MDS, while dyshaematopoiesis is less common. In addition, CD14 level increases significantly in children with JMML.