Abstract:

Objective　To study the clinical features of childhood acute lymphoblastic leukemia（ALL） with secondary myelofibrosis. Methods　The clinical data of 208 cases of newly diagnosed childhood ALL were analyzed retrospectively，who had difficulty in sucking the bone marrow fluid and underwent bone marrow biopsy from November 2001 to September 2012 in Hematology Hospital of Chinese Academy of Medical Sciences. The clinical characteristics and prognosis of childhood ALL patients with and without myelofibrosis were compared. Results　All the cases were separated into two groups，group A included 33 cases，who were diagnosed with ALL combined with bone marrow fibrosis confirmed by pathology；175 patients diagnosed with ALL without bone marrow fibrosis were enrolled into group B. From November 2001 to December 2007，we treated patients using CAMSBDH-ALL regimen，and from January 2008 to September 2012 we used CCLG-ALL 2008 regimen. In group A，42.4% of patients showed pancytopenia of peripheral blood. The 5-year overall survival rate（OS） was 64.3%. The 5-year OS was 58.3% in the patients treated with CAMSBDH-ALL regimen. In group B，15.6% of patients showed pancytopenia of peripheral blood，lower than that of group A. The 5-year OS was 85.0%，which was significantly higher than that of group A （P＝0.010）. The 5-year OS was 68.8% in the patients treated with CAMSBDH-ALL regimen，which was significantly lower than that of patients treated with CCLG-ALL2008 regimen （5-year OS being 87.2%）  in group B （P＝0.044）. Conclusion　Pancytopenia in childhood ALL with myelofibrosis are more common and with poorer prognosis than those without myelofibrosis. The overall survival of ALL patients without bone marrow fibrosis can be improved by modifying the treatment strategies，but the prognosis can’t be improved for those ALL patients with myelofibrosis.

Key words: acute lymphoblastic leukemia, childhood, myelofibrosis, secondary, clinical features

摘要：

目的　分析合并骨髓纤维化的儿童急性淋巴细胞白血病（ALL）患者的临床特点。方法　回顾性分析2001年11月至2012年9月中国医学科学院血液病医院初诊时骨髓抽吸困难并行骨髓活组织检查诊断的208例ALL患儿的临床资料。比较分析合并与未合并骨髓纤维化的儿童ALL的临床特点及预后。结果　208例患儿中，A组为经病理证实合并骨髓纤维化的患儿共33例，B组为未合并骨髓纤维化的患儿共175例。2001年11月至2007年12月采用CAMSBDH-ALL方案治疗，2008年1月至2012年9月采用CCLG-ALL 2008方案治疗。A组外周血细胞分析全血细胞减少者占42.4%。5年总生存率（OS）为64.3%，采用CAMSBDH-ALL方案治疗者5年OS为58.3%。B组全血细胞减少者占15.6%，明显低于A组。5年OS为 85.0%，明显高于A组患儿（P＝0.010）。B组患儿采用CAMSBDH-ALL方案治疗者5年OS为68.8%，低于采用CCLG-ALL 2008方案治疗者的5年OS（87.2%）（P＝0.044）。结论　合并骨髓纤维化的儿童急性淋巴细胞白血病较未合并骨髓纤维化者发病时全血细胞减少更常见，预后差，改变治疗策略可明显提高未合并骨髓纤维化者的总生存率，但未能改善合并骨髓纤维化者的总生存率。

关键词: 急性淋巴细胞白血病, 儿童, 骨髓纤维化, 继发性, 临床特点