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Clinical features and prognosis of children with acute lymphoblastic leukemia combined with myelofibrosis??An analysis of 208 cases

??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.