Relationship of Blimp-1 Hypoexpression with Pathogenesis of Aplastic Anemia / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To study the relationship of Blimp-1 hypoexpression with abnormality of Treg level and pathogenesis of aplastic anemia (AA).</p><p><b>METHODS</b>The mouse model with AA was established by adminis tration of IFN-γ combined with busulfan. The samples were collected at different day establishing AA model, and the spleen Treg number was detected, the Treg cells were sorted and expression level of prdm-1 was detected.</p><p><b>RESULTS</b>The number of Tregs in mice with AA was lower than that in control mice, moreover, the level of Treg decrease positively correlated with the AA severity (r=0.805), the higher the expression level of prdm-1, the higher the ratio of Treg/lymphocytes, showing positive correlation between them (r=0.548).</p><p><b>CONCLUSION</b>Blimp-1 expression may promote the proliferation and differentiation of Treg. The hypoexpression of Blimp-1 mediates the pathogenesis of AA and promotes progression of AA through reducing the proliferation of Treg, and decreacing the number of Treg.</p>

Clinical Charcteristics and Prognostic Analysis of 28 cases of Pediatric Myelodysplastic Syndrome / 中国实验血液学杂志

OBJECTIVE@#To analyze the clinical features and prognosis of 28 children with myelodysplastic syndrome (MDS) and to screen the high risk factors affecting the prognosis so as to provide the new ideas for standard of clinical diagnosis and therapy.@*METHODS@#The clinical data of 28 children with newly diagnosed MDS treated in our hospital from March 1994 to July 2016 were analyzed retrospectively, the features of disease onset and the results of laboratory examination were summarized, all MDS children were followed up, the prognosis and the high risk factors affecting the prognosis were evaluated.@*RESULTS@#In all 28 MDS children, the ratio of male to female was 1.8∶1, the incidence of MDS was observed in boys, while the low incidence of MDS was found in older children. The clinical manifestations were mainly the decrease of three series blood cells in 16 cases (57.14%), other cases presented simple anemia (7.1%), simple thrombocytopenia (7.1%), neutropenia with anemia (14.29%), and anemia with thrombocytopenia (14.28%).The bone marrow image showed mainly hyperplasia (82.14%), and the pathological hematopoiesis, moreover the manifistation of pathological hematopoiesis was different in forma and degree; the bone marrow biopsy showed the typical abnormal localization of immature precursor(ALIP) accepted for 33.33%; the chromosome karyotype detection showed the detected rate of chronosome abnormality was 41.18%. The median follow-up time was 1.75 years. 5 children with MDS received the hematopoietic stem cell transplantation (HSCT), among them 1 dead and 4 maintained CCR; Out of other 23 patients no-received HSCT, 7 cases given up treatment after confirmed diagnosis, 16 cases received the chemotherapy (2 cases given up treatment after CR, 5 cases transformed into AML, 3 cases relapsed, 3 cases maintained CCR), 11 cases dead, 9 cases failed to be followed up. The 5-years OS rate and EFS rate in all patients were predicted as (38.2±11.3)% and (35.3±11.3)%,respectively, among them, the OS and EFS rates of patients received the HSCT allo superior to those of patients did not received HSCT ［(80.0±17.9）% vs.(22.8±11.5）%］ (P0.05).@*CONCLUSION@#The children MDS is rare and easy to be misdiagnosis, moreover displays more high heterogeneity and poor prognosis, thereby the early diagnosis is crucial, in addition, the system of prognosis evaluation is imperative to be perfected. The HSCT may be the effective method for curative treatment of childhood MDS.

Clinical analysis of surgical treatment of tetralogy of Fallot for in infancy / 中国基层医药

Objective To sum up the therapeutic results of 125 cases of tetralogy of Fallot(TOF),and ex- plore the optimal time and risk factors of opration,as well as perioperative management.Methods One hundred and thirth-one consecutive cases of TOF underwent corrective surgery.There were simple stenosis of infundibular portion in right ventricular outflow tract in 37 cases,stenosis of infundibulum and pulmonary valve in 14 cases,main pul- monary trunk and left/right pulmonary arteries stenosis in 74 cases,and pulmonary atresia in 5 cases.Autologousper- icardial conduit,or with waived were used for right ventricular outflow tract and right ventriculo-pulmonary artery connection.Other anomalies were corrected.Results The surgicalmortality was 4.0 %.The cause of death were se- rious low cardiac output syndrome(3 patients),respiratory function failure(1 patient),pericadial infection(1 pa- tient).Conclusion It is necessary to profonn corrective opration on younger TOF patients.Effetive prophylaxis and control of low cardiac output syndrome and pulmonary complication is a useful strategy.