ABSTRACT
Objective To explore the clinical features and prognosis of different PML_RARα fusion gene isoforms in acute promyelocytic leukemia (APL). Methods The clinical data of 78 patients initially diagnosed with APL in Fujian Medical University Union Hospital from February 2013 to July 2016 were collected. The clinical features and prognosis of different PML_RARα fusion gene isoforms were analyzed. Results There were 32 females (41%) and 46 males (59%) in 78 patients, with a median age of 40 years old (13-68 years old). The most common PML_RARα fusion gene was L type (48.7%, 38/78), followed by S type (46.2%, 36/78) and V type (5.1%, 4/78). The patients with white blood cell count more than 10×109/L (high_risk) occurred mostly in S type (61.1%, 22/36), compared with V type and L type, and there were statistically different (χ 2 ＝ 7.683, P < 0.05). A total of 78 patients included 8 cases (10.2%) of combined CD34 positive, 17 cases (21.8%) of combined FLT3_ITD mutation, 12 cases (15.4%) of combined DNMT3A mutation and 9 cases (11.5%) of additional chromosomal abnormalities. There were no significant differences in CD34 positive, FLT3_ITD, DNMT3A, and the incidence of additional chromosomal abnormalities among the three different isoforms (P＞0.05). The most common occurrence of retinoic acid syndrome (RAS) during treatment was S type (21/36), while rare for L type and V type (χ2＝ 7.633, P< 0.05). There were no statistical differences in the complete remission (CR) rate and disease_free survival rate among the patients with different PML_RARα isoforms (P＞0.05). Conclusions The clinical characteristics of different PML_RARα fusion gene isoforms are different, including most_common L type, more_common V type and S type in high risk groups; complicated RAS is commonly found in S type during the treatment. And different isoforms have no effect on the CR and DFS rate.
ABSTRACT
Objective To evaluate the efficacy and safety of sequential treatment of newly diagnosed de novo acute myeloid leukemia (AML) patients with IA and low-dose HA combined with G-CSF regimens as remission induction therapy.Methods Fifty-seven patients with AML were enrolled,which marrow biopsy was hypocellular or active proliferation on the third day from the end of the first course with IA regimen.32 cases of them received the second course with low-dose HA combined with G-CSF regimen,compared with other 25 cases received the second course with another IA regimen.Clinical manifestations,blood count,blood biochemical parameters and bone marrow smears were measured during the courses.Results In study group,21 of 32 cases reached CR,4 PR,and 11 of 20 cases reached CR,2 PR in control group.Overall remission rate (ORR) was higher in study group than that in control group (78.1％ vs 52.0 ％,P =0.038).Both median duration of agranulocytosis and median time for PLT to reach 50×109/L from the lowest were shorter in study group than those in control group (9.5 d vs 28.0 d,U=32.5,P＜ 0.001; 11 d vs 19 d,U=193.0,P=0.001).Component transfusion,not only RBC but PLT,decreased in study group,compared with control group (8 U vs 16 U,U =206.5,P =0.002; 20 U vs 60 U,U =149,P ＜ 0.001).Median durable time of antibiotic intravenous injection was shorter in study group than that in control group (14 d vs 21 d,U=249.5,P=0.015).Visceral hemorrhage rate reduced in study group,compared with control group (x2 =3.90,P =0.048).Conclusion IA and low-dose HA combined with G-CSF regimens sequential treatment as remission induction therapy for newly diagnosed de novo AML patients is effective and well tolerated.