ABSTRACT
Objective To evaluate the efficacy of different treatment regimens for children with acute promye-locytic leukemia (APL)with positive PML -RARa fusion gene.Methods Thirty -two newly diagnosed APL patients were included in this study,treated either with all -trans -retinoic acid (ATRA)and chemotherapy (CT)(group A) or with ATRA and arsenic trioxide (ATO)(group B).Clinical situation and clinical efficacy were analyzed in patients in different groups.They were also separated into low risk group,intermediate risk group and high risk group according to different risk criteria.Clinical characteristics,complete remission,long -time survival and urine arsenic concentra-tion were analyzed and compared.Results (1 )Fourteen of 1 5 patients (93.3%)in group A achieved hematological complete remission (HCR)with a median time of 38 days (28 -63 days).Sixteen of 1 7 patients (94.1 %)in group B achieved HCR with a median time of 29 days (1 0 -42 days),which was significantly shorter than group A,and there was a significant difference between 2 groups(t =3.53,P =0.002).(2)The 5 -year event -free survival (EFS)of group A and group B was (60.0 ±1 2.6)% and (81 .9 ±9.5)%,respectively;the 5 -year EFS of group B was almost 20% higher than group A;while there was no significant difference between the 2 groups(χ2 =1 .1 5,P =0.28).The 5 -year overall survival (OS)of group A and group B was (72.2 ±1 1 .9)% and (94.1 ±5.7)%,respectively,the 5 -year OS of group B was almost 20% higher than group A;while there was no significant difference between the 2 groups(χ2 =2.88,P =0.1 6).(3)The 5 -year EFS of low plus intermediate group and high risk group patients was (74.0 ±1 0.1 )% and (64.8 ±1 4.3)%,the 5 -year EFS of low plus intermediate group was almost 1 0% higher than high risk group,but there was no significant difference between the 2 groups(χ2 =0.1 4,P =0.71 ).The 5 -year OS of low plus intermediate group and high risk group patients was (84.7 ±8.1 )% and (71 .3 ±1 4.1 )%,the 5 -year OS of low plus intermediate group was almost 1 0% higher than high risk group,while there was no significant difference be-tween the 2 groups(χ2 =0.36,P =0.55).(4)ATO related side effects were mild,including abnormal liver tests and e-lectrocardiogram,but were invertible after supportive therapy.At the end of each chemotherapy course,the urine arsenic concentration remained low and no chronic arsenic toxicity or second malignancies were found during the follow -up period.Conclusions The ATRA plus ATO regimen is a promising and better treatment for childhood APL with positive PML -RARa fusion gene compared with conventional chemotherapy.It was necessary to take risk stratification in APL patients.
ABSTRACT
Objective To assess the efficacy of combination therapy with all-trans-retinoic acid (ATRA ) and arsenic trioxide (ATO) in treating patients with acute promyelocytic leukemia (APL) .Methods A retrospective study was conducted to evaluate the efficacy of combining ATO with ATRA based induction therapy ,followed by 3 courses of consolidation chemotherapy and 2-year sequential ATO/ATRA maintenance therapy in newly diagnosed APL ,and the efficacy between high risk group and low/inter-mediate risk group ,also between different PML-RARa isoform sub-group were compared .Results In high risk group and low/in-termediate risk group ,the complete remission(CR)rates were 70 .0% and 96 .9% (P=0 .04) ,respectively ;the 3 years overall sur-vival rates(OS) and disease free survival rates (DFS) were(70 .0 ± 14 .5)% ,(96 .9 ± 3 .1)% ,P= 0 .01 and(66 .7% ± 19 .2)% , (93 .8 ± 6 .1)% ,P=0 .08 ,respectively .In BCR1 group and BCR3 group ,the CR were 78 .6% and 95 .6% (P=0 .14) ,respectively ;the rates of 3 years OS and DFS were(95 .7 ± 4 .3)% ,(78 .6 ± 11 .0)% ,P=0 .18 ,and(92 .9 ± 6 .9)% ,(87 .5 ± 11 .7)% ,P=0 .24 , respectively .Conclusion The results indicate that ATO based first-line protocol is highly effective for treatment of newly diagnosed APL ,especially for the PML-RARa BCR3 isoform APL .
ABSTRACT
The laboratory systems for chromosomal analysis or the detection of fusion genes are generally not available in Indonesia. Therefore, bone marrow (BM) morphological analysis should be developed and applied to get an accurate diagnosis. In this study the BM smears of eight (8) cases of acute myeloblastic leukemia (AML) which had already been known to have t(8;21)(q22;q22), were morphologically evaluated in order to find out the characteristics, which might be used to predict t(8;21)(q22;q22) or the presence of AML1-ETO(MTG8) fusion gene. All of the cases belonged to AML-M2. The morphological characteristics, indicative of t(8;21) were pink colored cytoplasm in mature neutrophil (75%), neutrophilic myelocytes or metamyelocytes without granules or with scarce granules (2.3%), eosinophilia (eosinophilic myelocytes and metamyelocytes) (above 5%), myelocytes with abundant granules 8.5%, and low percentage of type I blasts (below 10%). These characteristics were not observed in AML-M2 cases without t(8;21) or AML1-ETO(MTG8). The myelocytes with abundant granules have not been described so far, while other characteristics were in line with the findings of Nakamura et al (Leukemia 1997;11:651-55).