Comparison In Clinical Characteristics Of Acute Promyelocytic Leukemia With Different PML/RARA Isoforms

Objective:To analyze and compare the clinical characteristics of acute promyelocytic leukemia(APL)with different promyelocytic leukemia(PML)/retinoic acid receptoragene(RARA)isoforms.To discuss the relationship between clinical characteristics and prognosis of APL with different isoforms.Method:Choose 51 newly diagnosed acute promyelocytic leukemia(APL)patients in the hematology department of Shaanxi province people's hospital from 2010.02 to 2016.08 as the research object.Detect the three different isoforms of PML/RAR ?,and then group the patients into bcr1(long type,L)?bcr2(mutant type,V)and bcr3(short type,S).Gather and regulate the clinical materials.Analyze and compare the characters and prognosis of APL with different PML/RAR? isoforms.Results:1.The most common isoform of PML/RAR ? is L(30 cases,58.8%),then the subtype of S(18 cases,35.3%),and the most rare isoform is V(18 cases,35.3%).The distribution of inverse proportion of Shaanxi province people's hospital is different to Latin America.2.The clinical symptoms of APL are fever,fatigue,bleeding.Splenomegaly,superficial lymph node enlargement and sternal tenderness less happened.3.The bleeding proportion of V(33.3%)is less than L(43.3%)and S(66.7%),but P>0.05;The activated partial thromboplastin time of V is(20.95 s)shorter than L(28.6s)and S(27.55 s),and P(=0.049)<0.05;The D-dimer of S is(6.405mg/L)less than L(18.27mg/L)and V(21.78mg/L),P(=0.014)<0.05.4.FMS tyrosinase 3 internal tandem more often happens in subtype S(27.78%)than in L(3.33%)and V(0),P(=0.043)<0.05.5.The complete response times of bone marrow of L,V,S respectively are 25.5days,28 days,28 days,P>0.05.6.No 1 loss to follow-up of the 51 patients,and the middle follow-up time is 24 months.There are 4 patients relapse during the follow-up,the disease free survival times of L?V?S are 47 months?46 months and 54 months,the Log-Rank test P(=0.134)>0.05 in the three isoforms.Conclusion:The clinical featrues of different PML/RAR?subtype is not the same:The subtypes of L is more common in the fusion gene of PML/RAR?;The blooding occurrence rate of V is lowest;There are more FLT3/ITD in subtype S;ATRA,ATO and anthracycline-bassed chemotherapy can significantly increase the OS rate and DFS rate of APL.The prognosis of the three subtypes APL is basically the same.There was nosignificant difference between different subtypes of clinical features and prognosis.