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Analysis Of Clinical Characteristics And Prognosis Of Trisomy 8 In Myelodysplastic Syndrome

Posted on:2018-08-28Degree:MasterType:Thesis
Country:ChinaCandidate:L L ZhengFull Text:PDF
GTID:2334330536979035Subject:Internal Medicine
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Objective: To explore the incidence,cytogenetics characteristics,clinical features and outcome of trisomy 8 in myelodysplastic syndrome.Methods: 295 diagnosed patients of MDS between January 2011 and December 2016 were enrolled in our hospital.In these investigated patients there were 60 cases with trisomy 8.Check the diagnosis and classification based on diagnosis and effedct appraisal of blood diseases.Myelodysplastic syndromes of World Health Organization(2016).The clinical features and prognosis of MDS patients with trisomy 8 were retrospectively analyzed.The student test was used to measure the continuous variables between two groups,the variance of the variables were non parametric test,?2 tests was used to measure the categorical variables between two groups.The survival and prognosis of MDS patients with trisomy 8 were analyzed using the Kaplan-Meier method,using the Log-rank test and the COX proportional hazard model for the prognosis of single factor and multivariate factor analysis,all statistical significance were on basis of 0.05.Results:(1)There were 199 cases of abnormal chromosome detection in 295 MDS patients(67.46%).Trisomy 8 was found in 60 patients,representing 20.34% of all patients and 30.61% of patients with an abnormal karyotype,including 30 cases of isolated trisomy 8,the other 30 cases combined with other chromosomal abnormalities,the most common is the merger of 7q-,accounting for 16.49%.(2)The most common type of the WH0 2016 was MDS-MLD,accounting for 56.67%.(3)The most common of IPSS were Int-1 and Int-2(53.3%,26.7%);The most common of IPSS-R were high-risk,extremely high risk(30%,35%).(4)The group of patients with onset of anemia,7 cases of hepatomegaly,11 cases of splenomegaly,6 cases of enlarged superficial lymph nodes;the main clinical manifestation of two or three lineage cytopenia.Macrocytic anemia was the most common in MDS patients with isolated trisomy 8,accounting for 73.33%.9 cases with immunological abnormality;multilineage dyshaematopoiesis,degree of bone marrow hyperplasia to active.(5)In this study,5 MDS with trisomy 8 patients complicated with abnormal tetraploid,the median survival time was 29 months(range from 21 to 52.5 months).(6)5 cases of gene mutation detection,1 cases died with EVI1,DNMT3 A,PHF6,TET2 and ASXL1 gene mutation;1 cases died with TET2 and ASXL1 gene mutation;another 1 cases with ASXL1,U2AF1 and CSF3 R gene mutation,who is still alive;another 2 cases with no mutations.(7)In this study,8 patients received cyclosporine immunosuppressive therapy,including 6 cases of Int-1 in the risk stratification of IPSS and 2 cases of Int-2,6 cases of CR,1 cases of PR,1 cases of NR(overall response rate of 87.5%);4 patients who underwent allogeneic hematopoietic stem cell transplantation,3 patients are still survival,1 patients death with severe gastrointestinal GVHD.(8)The prognosis of MDS patients with trisomy 8 plus ?two aberration was better than trisomy 8 plus ? three aberration(P<0.05).No significant prognosis between isolated trisomy 8 and trisomy 8 with a single abnormal karyotype;no obvious relationship between trisomy 8 clone proportion and prognosis;patients with <5% bone marrow blasts and isolated trisomy 8 have the same prognosis as those with <5% and a normal karyotype;patients with ? 5% bone marrow blast have a shorter survival if an isolated trisomy 8 is found than if they have a normal karyotype.(9)Platelet count <100×109/L,Bone marrow blasts ?5% and trisomy 8 plus ?three aberration were independent risk factors for poor prognosis of MDS patients with trisomy 8.(10)The median survival time was 15 months of MDS patients with trisomy 8.There were 9 patients who transformed into acute myeloid leukemia with median transforming time of 4.2 months,the rates of blanching was 15%.1 cases of MDS patients with isolated trisomy 8 turned into tetrasomy 8,died after 4.3 months of follow-up.1 cases of MDS-EB2 patients transformed into M5,he accepted allogeneic hematopoietic stem cell transplantation and kept disease free survival.Conclusion:(1)Trisomy 8 was the most common cytogenetic abnormality in MDS patients in our center,which accounted for 20.34%.(2)The elderly was the most common,anemia was found in majority patients.Multilineage cytopenia,Multilineage dyshaematopoiesis,MDS-MLD were the most common.(3)Macrocytic anemia was the most common in MDS patients with trisomy 8.(4)The MDS patients with trisomy 8,who were combined with tetraploid abnormality had a long survival time and a good prognosis.(5)In intermediate group,MDS patients with trisomy 8 received immunosuppressive therapy had good effect.(6)Isolated trisomy 8 should be considered in the intermediate cytogenetic risk group,MDS patients with trisomy 8 who had more than 5% of bone marrow blast,platelet count <100×109/L and plus ?three aberration had a poor prognosis.(7)MDS patients with trisomy 8 had lower rates of blanching,and had better treated with allogeneic hematopoietic stem cell transplantation as soon as possible.
Keywords/Search Tags:Myelodysplastic syndrome, trisomy 8, prognosis
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