The molecular basis of myelodysplastic syndromes (MDS) is revealed by whole genome sequencing or targeted gene sequencing in several large cohort of patients with MDS.About 90 ％ of MDS patients carry ≥ 1 oncogenic mutations,the consistently mutated genes are SF3B1,TET2,SRSF2,ASXL1,DNMT3A and RUNX1.The further studies of clonal architecture of MDS and the functions of the involved genes might considerably improve prognostication of MDS and more generally clinical decision-making in this field.

Highlights of myelodysplastic symdromes (MDS) in the 54th ASH annual meeting were reviewed.Next-Generation Sequencing has revealed the spectrum of gene mutations in MDS patients.Some new molecular and cytogenetic parameters for increasing the number of responding patients and to further improve the durability of response to hypomethylating agents were comfirmed.

The novel second or next-generation sequencing (NGS) technique enables comprehensive mutational profiling analysis in patients with myeloproliferative neoplasms (MPN).JAK2,MPL and CALR gene mutations are successfully introduced into diagnostic practice,mutations in genes may impact outcomes such as survival and risk of leukemic transformation,therefore,molecular mutation analysis is ready for use in all patients with suspected MPN.

During the past few years, European consensus on grading bone marrow fibrosis, the Dynamic International Prognostic Scoring System (DIPSS) for primary myelofibrosis(PMF) and response criteria for PMF were developed, and some new drugs, such as the immunomodulator drugs (IMiDs) and JAK2 inhibitor are available for PMF. The recent advances were summarized and future issues were discussed.

Objective To improve the understanding of systemic mastocytosis(SM) with associated clonal haematological non-mast-cell lineage disease (SM-AHNMD). Methods The clinical and laboratory features, treatment and prognosis of a rare case of SM-AHNMD was reported and the related literatures were reviewed. Results As the second most common subtype of SM, SM-AHNMD mainly has multifocal dense infiltrates of mast cells detected in sections of bone marrow and/or other extracutaneous organs, and some special cytology or biochemical markers(toluidine blue staining,tryptase,CD117,CD25 and/or CD2) results are positive. C-kit mutations are found in most adult patients. SM-AHNMD also has clinical characters of other clonal haematological non-mast-cell lineage disease (HNMD), such as myelodysplastic syndrome,myeloproliferative neoplasms, acute myeloid leukemia. Conclusion SM-AHNMD meets criteria for SM and other HNMD. Treatment for SM-AHNMD is mainly about the therapy of HNMD, and the prognosis greatly depends on the accompanying hematological disorder.There is no special treatment for SM,and the treatment should be directed at symptomatic control.

Objective To observe clinical efficacy of recombinant human erythropoietin(rhu-EPO)in sepsis with anemia.Method From June 2005 to December 2006,sixty sepsis with anemia patients in the Second Affiliated Hospital of Tianjin Medical University were divided into control group(n=30)and theatment with rhu-EPO group(n=30).The exclusion criteria was:renal imufficiency patients needing blood purification,patients with hemorrhage of digestive tract,and patients who used rhu-EPO before.Patients in the rhu-EPO group were hypodermically injected with rhu-EPO at 48 hours after admission,6000 units par time,and continued every other day for two weeks.When the hemoglobin Was lower than 80 g/L,patients received red blood cell(RBC)transfusion or received other treatment decided by doctor.The data were expressed as(x±s),and were analyzed using Student's t test and Fisher's exact test with SPSS 11.5.A P valLie less than 0.05 indicated statistical significance.Results There Was no significant difference on hemoglobin between the two groups before treatment (P＞0.05).After treatment,the hemoglobin of patients in rhu-EPO group was higher than that in control group [(105.87±11.48)g/L vs.(91.23 ±19.89)g/L,P＜0.01].The units of RBC transfusions was less in patients of rhu-EPO group compared with control group[(2.0 ±0.47)U vs.(2.63±0.43)U,P＜0.01].There was no significant difference on mortality between two groups(P＞0.05).Conclusions In sepsis with anemia patients,administration of rhu-EPO every other day can increase red blood cell level and reduce RBC transfusion.

Objective]To investigate the thought of manipulating The Three Blood Statsis Removal Decoctions(Taohe Chengqi Decoction, Di Dang Decoction, Di Dang Pills) of ZHANG Zhongjing.[Method]Collect and analyse the explanations of the scriptures involving the Three Blood Statsis Removal Decoctions of ShangHanLun interpreters from past to nowadays, combine with the theory said in Huang Di Nei Jing, knowledge of exegetics and personal view, in order to summarise the original intention of manipulating the Three Blood Statsis Removal Decoctions by ZHANG Zhongjing. [Result] The main purpose of Taohe Chengqi Decoction is to purge heat, since heat is the main reason of stagnation of blood. It applies to the preliminary stage of combination of heat and blood clot. The main purpose of Di Dang Decoction is to attack the rigid blood-heat stagnation in lower-energizer, it is applied to the late stage of combination of heat and blood clot. Di Dang Pill is the milder prescription of Di Dang Decoction, which is applied to the milder situation of blood-heat combination. [Conclusion]Comparing the Three Blood Statsis Removal Decoctions,“ Superficial”and“ deep”words should be used to differentiate the application of Taohe Chengqi Decoction and Di Dang Decoction, while“ light”and“ severe”words should be used to differentiate that of Di Dang Decotion and Di Dang Pills.

Objective To study the frequency and characteristics of secondary monoclonal gammopathy of undetermined significance(sMGUS) in multiple myeloma (MM),and analyze the impact on survival.Methods The data of 515 patients with MM admitted were analyzed retrospectively.73 cases of patients underwent stem cell transplantation and 442 patients received thalidomide or bortezomib based chemotherapy.Immunofixation electrophoresis(IFE) and clinical characteristics were respectively analyzed,and the comparison of survival between sMGUS group and non-sMGUS group was performed.Results Thirty-five cases (6.8 ％) of myeloma patients with sMGUS were found in all patients.The incidence of sMGUS after hematopoietic stem cell transplantation treatment is significantly higher than that of receiving chemotherapy (19.2 ％ versus 4.8 ％,x2 =20.587,P =0.002).The CR rates of sMGUS group and non-sMGUS group were 45.7 ％ (16/35) and 14.3 ％ (59/480) (x2 =22.961,P ＜ 0.001).The median survival time of patients with sMGUS was much prolonged compared with the control cohort (42.0 versus 14.0 months,P ＜ 0.001).However,when the analysis was restricted on patients underwent stem cell transplant,patients with sMGUS had a negative impact on outcome,and the median overall survival was 30.8 and 39.3 months (P =0.002).Conclusion The sMGUS may be attributed to either immune reconstitution or immune system dysregulation after highly immunosuppressive therapy.The incidenceof sMGUS after auto-SCT treatment is higher than chemotherapy.The sMGUS group has the higher response rate and longer survival.But for auto-SCT treatment patients,sMGUS may be not a good prognostic factor.

To investigate the relationship of between p21wAF1 and PCNA expression and the patients' prognosis in breast carcinoma. MethodExpression of p21wAF1 and PCNA was determined by S-P immunohistochemical technique in 152 patients with breast carcinoma. ResultsPositive immunostaining of p21WAf1was detected in 61(40.1% ) primary breast carcinoma, in which 43(70.5% ) were low expression, and 18(29.5% ) over expression of 152 PCNA positive cases, 69(45% ) expressed with low index and 83(55% )expressed with high index. p21wAf1 expression level was correlated with tubule formation (γs＝ 0.341, P＜0.01 ),nuclear atypia(γs=0.516, P＜0.01), mitosis(γs=0.351, P＜0.01)and histological grade(γs=0.415, P＜0.01)in breast cancer tissue. Univariate analysis demonstrated less disease-free survival in patients with higher p21WAF1 (x2 = 6.92, P＜ 0.01 ) and PCNA (x2 = 5.14, P＜0.05)expression than in those with lower expression. The survival term of the patients with p21WAF1 -/PCNA low index, p21wAF1 +/PCNA low index, p21WAF1 +/PCNA high index and p21WAF1 -/PCNA high index groups was successively shortened. Conclusions( 1 ) Combined detection of p21wAF1/PCNA is helpful to judge the proliferating activity of the tumor cells and the patients prognosis. (2) Different expression level of p21WAF1in breast carcinoma is probably related the different regulating directions.

Objective To analyze the correlation of visual acuity and metamorphopsia with foveal morphology before and after vitrectomy with internal limiting membrane peeling (ILMP) in idiopathic macular epiretinal membrane (IMEM) eyes.Methods This is a retrospective case series of 47 IMEM patients (49 eyes).All the patients underwent 25G pars plana vitrectomy with IMEM removal and ILMP.The best corrected visual acuity (BCVA) was measured using the international standard visual acuity chart,and the results were converted to the logarithm of the minimum angle of resolution (logMAR) visual acuity.The severity of metamorphopsia was measured using M-charts.The central macular thickness (CMT),inner nuclear layer thickness (INT),inner retinal layer thickness (IRT),outer retinal layer thickness (ORT),the status of ellipsoid zone (EZ) were assessed with spectral-domain optical coherence tomography at baseline and each month postoperatively.The differences in BCVA,CMT,INT,IRT,ORT and status of EZ before and after surgery were analyzed,so did the correlations between these indexes at the same time.Results Compared with baseline,the postoperative BCVA was significantly increased (F=6.133,P＜0.001),but the M value,CMT,INT,IRT,ORT were significantly decreased (F=12.481,10.565,15.739,6.046,10.569;P＜0.001);the integrity of EZ was improved significantly (x2=12.309,P＜0.001).Preoperative BCVA was positively related to the CMT (r=0.720) and ORT (r=0.720,0.887;P＜0.05),while negatively related to preoperative integrity of EZ (r=-0.295,P＜0.05).The postoperative BCVA was positively related to the preoperative BCVA and postoperative CMT,ORT (r=0.774,0.754,0.842;P＜0.05),while negatively related to postoperative integrity of EZ (r=-0.676,P＜0.05).The preoperative M value was positively related to the preoperative CMT,INT,IRT,and ORT (r=0.931,0.668,0.840,0.637;P＜ 0.05).The postoperative M value was positively related to the preoperative M value and postoperative CMT,INT,IRT,and ORT (r=0.723,0.722,0.767,0.825,0.387;P＜0.05).Conclusions Vitrectomy with ILMP for IMEM can improve the visual acuity,metamorphopsia and foveal morphology.Both visual acuity and metamorphopsia correlate with foveal morphology.