Clinical Biological Characteristics and Prognostic Factors of Children with PhALL / 中国实验血液学杂志

ABSTRACT

OBJECTIVE@#To investigate the clinical biological characteristics of children with Ph ALL and the factors that affecting its prognostic.@*METHODS@#34 children with Ph ALL were selected retrospectively in the period from January 2006 to December 2017; the clinical biological characteristics, clinical efficacy for short-term and survival time for long-term were recorded and the related factors that affecting the clinical efficacy for short-term and survival time for long-term were evaluated.@*RESULTS@#The median overall survival time was 16.5 months and the cumulative OS rates for 2 years and 5 years with followed-up were separately (61.57±7.09)%, (50.75±8.22)%. The median progression-free survival time was 13.5 months and the cumulative progression-free survival rates for 2 years and 5 years with followed-up were separately (54.49±6.77)%, (48.77±7.42)%. The early treatment response of patients with myeloid antigen expression were significantly lower than the patients without myeloid antigen expression (P＜0.05). The CR rate in one treatment course of patients with good response for early treatment response group were significantly higher than the patients with poor response for early treatment response (P＜0.05). The CR rate in one treatment course of the patients with TKI addition in induction therapy period group were significantly higher than the patients with chemotherapy used alone（P＜0.05）. The OS rate and PFS rate of patients in chemotherapy + TKI + allo-HSCT and chemotherapy + TKI group were significantly higher than the patients with chemotherapy used alone group（P＜0.05）. Univariate analysis showed that the factors affecting OS rate of children with Ph ALL for 2 years with followed-up included baseline WBC count level, LDH level, distances between lower hepatic margin and costal margin, distances between lower splenic margin and costal margin, combined myeloid antigen (+), early treatment response, FCM-MRD status after one treatment course, BCR-ABL status after one treatment course and TKI application (P＜0.05) and the factors that the affecting PFS rate of children with Ph ALL for 2 years with followed-up included LDH level, distances between lower hepatic margin and costal margin, distances between lower splenic margin and costal margin, combined myeloid antigen (+), early treatment response, FCM-MRD status after one treatment course, BCR-ABL status after one treatment course and TKI application (P＜0.05). Multivariate analysis showed that the distances between lower splenic margin and costal margin 3 cm were independent risk factors on OS rate and PFS rate of children with Ph ALL (P＜0.05).@*CONCLUSION@#Children with Ph ALL possess unique clinical and biological features. The prognosis of patients with chemotherapy used alone is more poor, and the combination of chemotherapy and TKI can effectively increase the survival benefit; patients with Ph ALL combined with myeloid antigen (+) shows a poor early treatment response. while the distances between lower splenic margin and costal margin 3 cm are independent risk factors on clinical prognosis of children with Ph (+) ALL.