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.

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.

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.

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 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 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.

Aim To investigate whether there is IL-12 signaling pathway through lck/P38/c-jun in splenic cells obtained from lupus mouse and its effect on splenic cells. Methods Mice with graft versus host disease were used as lupus nephritis model. Activity of Lck tyrosine kinase, p38 phosphorylation and mRNA expression of c-jun in splenic cells were determined by autoradiography, Western blot and Northern blot, respectively. Results There were higher levels of Lck activity, p38 phosphorylation and c-jun expression of IL-12-stimulated splenic cells from lupus model when compared with that observed in similarly treated splenic cells from normal control. The Lck activity and p38 phosphorylation were almost inhibited by Lck inhibitor PP1, on the other hand, p35 specific inhibitor SB203580 decreased phosphorylation of p38. In addition, expression of c-Jun was also inhibited by PP1 or SB203580 although splenic cells were stimulated with IL-12. Conclusion Aberrant murine splenic cell, IL-12-me-diated intracelluar signaling pathway through lck/p38/c-Jun were involved in immunologic damage.

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.

Objective To evaluate the safety and effectiveness of vitrectomy combined with internal limiting membrane (ILM) tamping on macular hole and retinal detachment (MHRD) in highly myopic eyes.Methods 23 patients (23 eyes) were retrospectively reviewed,who were diagnosed as MHRD through examination of the ocular ftmdus,optic coherence tomography (OCT) and B-mode ultrasonography.There were 5 males (5 eyes) and 18 females (18 eyes).The mean age was (62.35 ± 8.28) years.The mean course of disease was 1.1 months.The logarithm of the minimum angle of resolution (logMAR) best-corrected visual acuity (BCVA) was 2.31 ± 0.72.The mean axial length was (28.66 ± 1.99) mm.All patients underwent 23G microincision vitrectomy.After vitreous gel and cortex were gently resected,the ILM around the edges of the macular hole was stained with indocyanine green,and was folded and pushed to fill the macular hole gently.Then silicone oil or C3F8 gas tamponade was applied in 18 eyes and 5 eyes,respectively.The silicone oil was removed after 3 months.The follow-up was 6 months.The BCVA,macular hole closure,retinal anatomical reattachment were retrospectively observed,and were used to evaluate the safety and effectiveness of the surgery.Results At the 6 months after surgery,the logMAR BCVA was improved to 1.13 ± 0.38,the difference was significant (t=l 5.33,P=0.00).The postoperative macular hole closure rate and retinal anatomical reattachment rate were 100％.There were no ocular or systemic adverse events observed in all patients.Conclusion Vitrectomy combined with ILM tamping is an effective and safe treatment for the high myopic eyes with MHRD.