The Clinical And Laboratory Characteristics Of Refractory Cytopenia Uniparental Dysplasia

Objective:Elaborated clinical and laboratory features, diagnosis and prognosis of myelodysplastic syndrome- Refractory cytopenia uniparental dysplasia.Methods:(1)Collected outpatient and hospitalization of the clinical data of 75 patients with RCUD from January 2005 to October 2014.(2)Classified these patients with peripheral blood cell counts and bone marrow morphology respectively, analysis the correlation of them.(3) Mainly analysed the cytogenetic characteristics of these patients.(4)Analysed the features of these patients’ immune phenotype through flow cytometry,and then compared with the other subtypes of MDS.(5)Rated he patients with IPSS and IPSS-R scoring system respectively,compared the differences between the subtypes and the reported data. Reviewed the prognosis of MDS.Results:(1)Compared morphological classification and cell counts showed that erythropoietic dysplasia was accompanied by isolated anemia in 48%,dysgranulopoesis was accompanied by isolated neutropenia in 40%, and megakaryocytic dysplasia was accompanied by isolated thrombocytopenia in 30%.(2)There were 20 patients with abnormal karyotype. Among the abnormal karyotype,+8 accounted for 25%,-Y accounted for 15%, dup(1) accounted for 10%,20q- accounted for 10%, and del(11q),der(22),ins(10;3),t(7;21) accounted for 5%respectively.(3)The cells group on CD45 / SSC dot plot were scattered,the expression of monocytes CD45 was decreased,CD34 was increased,B precursor cells were decreased.The express curve of CD33/CD13 was disordered,the expression of CD10 was decreased,CD71 was increased obviously.The expression of CD11b/CD16 and CD13/CD16 was abnormal.(4)According to the IPSS, the proportion of low-risk, intermediate risk-1and intermediate risk-2 of the RA patients was 11(79%),3(21%) and 0(0%),the proportion of the RN patients was 11(58%),8(42%) and 0(0%),the proportion of the RT patients was 9(64%),5(36%) and 0(0%).According to the IPSS-R, the proportion of very low-risk, low-risk,intermediate risk and high risk of the RA patients was1(7%),10(71%,2(14%) and 1(7%),the proportion of the RN patients was12(63%),4(21%),2(11%) and 1(5%),the proportion of the RT patients was1(7%),11(79%),2(14%) and 0(0%).Conclusion:(1)The peripheral blood cell counts and bone marrow morphology of these RCUD patients were largely in line with the WHO classification standards,but there was no strong correlation between the them.(2)In addition of the common abnormal karyotype,such as +8,-7/7q-,20q- and-Y,these was a variant—t(7;21)(p21;q22) from t(8;21)(q22;q22).The patient is long-term disease-free survival after autologous peripheral blood hematopoietic stem cell transplantation.(3)The majority of these patients could be detected abnormal immune phenotypic of granulocyte,mononuclear and B lymphocyte,but these was no particularity with the other subtypes of MDS.(4)According to the IPSS,the majority of these patients were belong to low-risk group.But according to the IPSS-R,the majority of RA and RT patients were belong to low-risk group,these was a patient of RA belong to high risk group,which was belongto intermediate risk-1(IPSS).The plenty of RN patients were belong to very low risk group,one of them belong to high risk group.