| Background and ObjectiveAtypical chronic myeloid(aCML)is a rare hematopoietic stem cell disorder,with overlapping features of myelodysplastic syndormes and myeloproliferative neoplasms.aCML is categorized as a myelodysplastic/myeloproliferative neoplasms(MDS/MPN)according to the World Health Organization classification of tumors of hematopoietic and lymphatic tissues.The aim of this study was to investigate the clinical and laboratory characteristics of 13 aCML patients diagnosed in our institute since 2007.MethodA retrospective study on the clinical and laboratory characteristic of 13 aCML cases was performed.according to WHO 2008 classification.Comparisons between continous variables were performedusing the Mann-Whitney U test.Comparisons between categorical variabled were performed using the Chi-square tests.ResultOf the 13 aCML patients,the median age was 56(31-75)years with a male predominance(9/13).The median hemoglobin was 109(30-150)g/L,with median MCV 93.5(76.2-115.8)fl,median WBC 24.8(4-77.6)×109/L,median PLT 68(10-)×109/L,median neutrophil proportion 72(32-87)%,median AMC 0.52(0.1-5.74)×109/L,median ALC 1.9(0.41-8.2)×109/L,median AEC 0.13(0-8.55)X 109/L.7 of 13 patients accompanied with splenomegaly,with 4 of them LCM ≥10cm.2 of 13 patients harbored cytogenetic abnormalities,with 47,XY,+?8[20]and 47,XX,+8[15]each.4 of 13 patients were conducted with next-generation sequencing,one carried TET2、one carried U2AF1、SETBP1,one carried U2AF1、ASXL1,another harbored U2AF1、CEBPA and SETBP1.Bone marrow smears showed 10 of 13 cases displayed with dysgranulopoiesis,such as decreased granules,pseudo Pelger-Huet,irregular hypersegmentation,megaloblastic changes etc;7 of 13 displayed with dyserythropoiesis,such as multinuclearity,nuclear budding,megaloblastic changes,karyorrhexis,H-J body etc.9 of 13 displayed with megakaryocyte dysplasia,such as single round nucleus,double round nucleus,multiple round nucleus ect.ConclusionCompared with previous data,our patients showed younger age,lower white blood cells,lighter anemia and more severe thrombocytopenia at diagnose,this difference may due to the limitation of sample size. |