Clini Calcharacteristic And Prognosis Of IKZFL Deletion In Pediatric Acute Lymphocytic Leukemia Were Retrospectively Analyzed

Objective To study clinical biological characteristics and the the prognosis ofIKZF1deletion of children in pediatric acute lymphocytic leukemia.Method Retrospectively,we selected181cases of children with ALL from thedifferent clinical risk classification.IKZF1gene promoter and exons are missing accordingto AccuCopy which is Multiple gene copy number detection technology.Such asdetection of IKZF1gen e promoter or8exons of the lack of any a function sequence, thatrepresent the lack of IKZF1genes.We statistically analyzed IKZF1genetic flaws anddifferent function sequence spectrum in ALL children,what have clinical features andprognosis with chromosome, MRD, gene fusion BCR/ABL1and the MLL,and so on.Results1.The lack of IKZF1gene rate and the lack of different function sequence spectrum1.1IKZF1gene deletion rates181cases had already33cases of detection IKZF1gene in ALL children, loss rateof18.23%. Among them,83cases of high-risk group with25cases in children with IKZF1gene deletion, accounted for30.12%; In98cases of children with low in the intermediaterisk category8cases of IKZF1gene deletion, accounted for8.16%;27cases withBCR/ABL1fusion gene were12cases of IKZF1gene deletion, accounted for44.44%;49cases in children with recurrent,18cases of IKZF1gene deletion, accounting for36.73%.1.2. IKZF1gene deletion had different function sequence spectrumIn33cases of IKZF1gene deletion, we found that the promoter gene single copy allmissing:14cases (42.42%),4-7exons single copy is missing:11cases (33.33%), thepromoter and exon4to7pairs of missing copy:4cases (12.12%),2-3exons single copy ismissing:4cases (12.12%), of which has the highest percentage of lack of promotersequences. 2.IKZF1gene deletion and Clinical CharacteristicsAccording to chi-square, IKZF1gene deletion or not in children with different gendergroups (χ2was0.8, P0.442), prednisone pretreatment is sensitive or not (χ2was0.004, P0.624), the presence of MLL gene rearrangement group (χ2was0.355, P0.400), differentprognosis karyotype (χ2was8.000, P0.238),which are not statistically significantdifference between the groups. And the fusion gene BCR/ABL1(χ2was14.626, P0.001),and33days MRD>1x10^-4(χ2was14.645, P0.001), the difference has the obviousstatistical significance. According to Wilcoxon rank and inspection, two comparison,IKZF1gene deletion or not in age greater than2(Z was2.749, P0.006), the white bloodcells than50x10^9/L (Z is1.964, P0.049)，both the layered high-risk group (Z was1.993, P0.046) difference was statistically significant.3.Lack of IKZF1gene and the prognosis3.1According to IKZF1gene in181cases of children with ALL missing or not aredivided into groups and not missing, missing18.23%and81.77%respectively, itsfive-year survival rates were42.5%,66.5%, the Log-rank chi-square test value of3.970,P was0.046(P <0.05). Both survival difference statistical significance, missing group waslower than that in group without missing.3.2lack of IKZF1gene different function sequence spectrum’s relationship withprognosisIKZF1deletion spectrum is given priority to with lack of the promoter, the promoterof the lack of single copy genes and the4-7exons single copy is missing5years ofsurvival rate are14.0%and14.0%respectively, the Log-rank chi-square test value of0.226, P was0.634(P>0.05),5years of survival rate differences between groups have nostatistical significance.3.3lack of IKZF1gene and the recurrence relationsIn children with181cases already, lack the gene group of33cases,18cases ofrecurrence (54.50%),15cases without recurrence (45.50%), the gene is not missing groupwith148cases, including31cases of recurrence (20.95%%),117cases (79.05%%)without recurrence, the gene deletion recurrence with obvious differences between thegroups with and without missing (chi-square was15.429, P was0.000).33cases ofrecurrence of IKZF1gene in children with missing,18cases (54.50%),15cases (45.50%)without recurrence, its five-year survival rates were8.7%,93.3%, the Log-rank chi square test is12.019，and P was0.001.Conclusion1.This group of children with ALL IKZF1gene deletion rate was about18.23%, missing its sequence mainly include the lack of the promoter; Lack of IKZF1genedifferent function sequence spectrum is not the main factors influencing the prognosis ofchildren with ALL.2. Gender, prednisone pretreatment effect, MLL gene rearrangement and chromosomekaryotype is not the main factors influencing the lack of IKZF1gene or not; IKZF1genedeletion in33days MRD>1x10^-4, BCR/ABL1fusion gene was positive, white bloodcells than50x10^9/L，more than2years old,clearly in children with high incidence,risk stratification and both the high-risk group was significantly related factors.3.Lack of IKZF1gene is an important factor affecting ALL children with recurrent,also is the important factors affecting the survival rate, risk stratification factor may be asan independent.