Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disorder of hematopoietic stem cells due to acquisition of somatic mutations.Somatic mutations in phosphatidylinositol glycan class A (PIGA) account for intravascular hemolysis and other PNH manifestations,but the pathophysiology of clonal expansion of PNH cells cannot be elucidated clearly.PNH is closely related to aplastic anemia and myelodysplastic syndromes.Today,the gold standard for PNH is flow cytometry to detect the absence or severe deficiency of glycosylphosphatidylinositol (GPI)-anchored proteins on white and red blood cells.However,PNH diagnosed by phenotype is a group of heterogeneous disease in pathogenesis.Eculizumab,a first-in-class monoclonal antibody that inhibits terminal complement,is highly effective in stopping intravascular hemolysis and improving quality of life.Further research on the pathogenesis of PNH would be helpful to understand the underlying reasons for PNH phenotype cells in different patients,improve differential diagnosis and more targeted and specific therapy.Research progress in recent years will be reviewed in this article.

AIM:To explore the effect of leptin on the expression of degeneration-related genes in rat nucleus pulposus ( NP) cells and to detect the possible mechanism .METHODS:The normal NP cells isolated from SD rats were analyzed by immunochemistry and immunofluorescence for the collagen II and cytokeratin 19 expression.The NP cells were treated with leptin and/or interleukin-1β( IL-β).The mRNA expression of MMP-1, MMP-3, MMP-9, MMP-13, ADAMTS-4, ADAMTS-5, aggrecan and COL2A1 in the cells was detected by real-time PCR.Alcian blue staining and im-munochemistry were used to examine the expression of proteoglycan and collagen II .Activation of involved pathways was studied by Western blot .The inhibitors of the pathways were used to reveal the effect of these pathways on NP cells .RE-SULTS:The results of real-time PCR revealed that leptin alone up-regulated the mRNA expression of MMP-1, MMP-13, ADAMTS-4, ADAMTS-5 and COL2A1.The synergy of leptin and IL-βwas found in the increased expression of MMP-1, MMP-3 and ADAMTS-5.The NP cells treated with leptin showed less expression of collagen II .Both PI3K/Akt and JAK2/SATA3 pathways were activated by leptin , whereas only inhibitor of JAK 2/SATA3 pathway reversed the expression of MMP-1 and MMP-13.CONCLUSION:Leptin may promote catabolism in rat NP cells via JAK2/SATA3 pathways, which may be the mechanism mediating the association between obesity and intervertebral disc degeneration .

Objective To explore the effect of progressive active respiratory exercise on the pulmonary function and quality of life of middle-aged and elderly patients after surgery for carcinoma of the esophagus.Methods Forty post-operative middle-aged and elderly patients with carcinoma of esophagus were randomized into 2 groups.The patients in the control group were given conventional thoracic nursing,while those in the experimental group were given 3-step thoracic or abdominal breathing training,breath holding training and respiratory gymnastics training in addition.A lung function test,the 6-min walk test and a 36-item short form health survey (SF-36)were used to evaluate the subjects' pulmonary function,motor ability and quality of life after 3,6 and 12 months of treatment.Results Vital capacity (VC) increased 23％ after training.Forced vital capacity (FVC) increased 16％.Maximal ventilation volume (MVV) increased 15％ and 50％ of forced expiratory flow (FEFS0) and the diffusing capacity for carbon monoxide of the lungs (DLCO) increased 30％.Average distance in the 6-minute walk test (6MWD) lengthened from 320 m to 449 m,and the quality of life of the patients in the experimental group improved much more than that of the control group.The average pulmonary function and 6MWD of the control group did not improve significantly with only nursing care.Their average SF-36 scores for physical function,mental health,physical pain,mental role and comprehensive health did,however,improve significantly after treatment.The changes in their role limitation,social function and energy scores were not significant.Conclusion Early and progressive active respiratory exercise can ameliorate lung dysfunction after a thoracic operation,and improve motor ability and quality of life.

AIM:Toexploretheeffectofglucocorticoidonautophagyandsenescenceinthechondrocytes. METHODS:The collagen II in the normal chondrocytes isolated from the SD rats was checked.After stimulation with glu-cocorticoid, LysoTracker Red staining, MDC staining and Western blot were used to detect the level of autophagy in the chondrocytes.The mTOR pathway related molecules were investigated by Western blot.Cell senescence was analyzed by SA-β-gal staining.RESULTS:A dose-dependent increase in the number of autophagic vacuoles was observed in the dexa-methasone-treated chondrocytes, which was demonstrated by the LysoTracker Red and MDC staining.The expression of LC3-II and beclin-1 was increased by dexamethasone, especially in the cells treated with dexamethasone for 4 d.However, P62 expression was decreased.SA-β-gal staining showed that the percentage of cell senescence was increased by dexam-ethasone.Surprisingly, the cell senescence induced by dexamethasone was exacerbated by the autophagic inhibitor 3-MA. CONCLUSION:Autophagy induced by dexamethasone protects chondrocyte from senescence.The mTOR pathway may be involved in the autophagy activation.

Objective To describe the results of the Universal Newborn Hearing Screening (UNHS) in Beijing in 2010; and investigatethe factors related with the results. Methods The result of the UNHS investigated by 106 maternity hospitals were reported. These hospitalswere investigated with the self-made questionnaire. Results In 2010, 159888 infants (91.99%) were screened in 106 maternity hospitals inBeijing as the 1st stage UNHS. 10.32% of them failed OAE tests. 72.30% received the 2nd stage UNHS and 13.27% of them failed testsagain. The referral rate was 0.99% in the end. The screening rate of both the 1st and 2nd stage was more in the newborn with Beijing householdregistration than in those with other household registration, as well as the hospitals in urban than in rural areas. For the rate fail in the1st stage UNHS, it was more in hospitals in urban than in rural areas, with distortion product otoacoustic emission (DPOAE) than with transientevoked otoacoustic emissions (TEOAE), by the bed than in the special room, and screening only once than screening again. ConclusionThe quality of UNHS is as high as in the developed countries in the newborn with Beijing household registration. To further improvethe screening rate, it is important to focus on the newborn with other household registers and the hospitals in rural areas. The instrument, personnelqualifications, environment and operation need to be further standardized.

Objective To study the relationship between the six-minute walking test (6-MWT) and pulmonary function in the diagnosis course of chronic bronchitis converting into chronic obstructive pulmonary disease (COPD). Methods Pulmonary function was detected in 360 patients with chronic bronchitis.Based on the results and diagnosis standard of COPD by Chinese Medical Association in 2002, the patients were divided into COPD group and pulmonary function normal group.Tbe walking distance in 6-MWT (6-MWD) was measured, and the mean value of twice tests was calculated to study the correlation between 6-MWT and pulmonary function, and 50 healthy volunteers were selected as control group. Results Most 6-MWD in COPD group were less than 420 meters(95.95%, 142/148 ), but those in pulmonary function normal group were more than or equalled to 420 meters mostly (97.17%, 206/212 ). However in patients with the 6-MWD more than or equalled to 420 meters , pulmonary function indexes such as the ratio of forced expiratory volume in one second and forced vital capacity( FVC ), FEV_1%, maximal ventilatory volume(MVV)were all significantly greater than those in patients with 6-MWD less than 420 meters, and there were significant differences (P<0.05). Compared with chronic bronchitis, the results of 6-MWD in control group was more than or equalled to 420 meters, but there was no significant difference (P>0.05 ). Conclusions 6-MWT is simple and feasible. However, it is a better indicator of the patients pulmonary function. The test would be an effective selecting examination for chronic bronchitis patients who are not willing to do puhnonary function test.

Telomere and telomerase play important roles in protecting and maintaining the stability and integrity of chromosomes. Telomere and telomerase are closely related with multiple diseases, and their relationship with aging and tumors has become a hot topic in recent years. Relevant reports in the American Society of Hematology Annual Meeting in 2016 will be reviewed together with advances of telomere and telomerase in hematological diseases.

Although tyrosine kinase inhibitors (TKI) have profoundly improved prognosis of patients with chronic myeloid leukemia (CML), it still can not cure CML with high relapse rate when TKI is used alone. Except for effective development of TKI and precise application of the existing TKI,the exploitation of new therapy target, research on treatment for leukemia stem cells and cocktail targeted therapies also provide further inspiration for improving treatment and curing CML. This article will introduce the advances of therapy in CML.

Objective The aims of this study were to investigate the prognostic value of CEA and CA19-9 expression in advanced gastric cancer and the prognostic factors of advanced gastric cancer.Methods A total of 255 patients with advanced gastric cancer who underwent radical R0 radical surgery were enrolled in the Department of Gastrointestinal Surgery,Harbin Medical University Cancer Hospital from January 2010 to December 2010,and were divided into group A(CEA<2.19ng/mL and CA19-9<10.78U/mL),group B(CEA≥2.19ng/mL or CA19-9≥10.78U/mL)and group C(CEA≥2.19ng/mL and CA19-9≥10.78U/mL).These groups were analyzed the differences in gastric cancer patients with clinical pathology and survival,and prognostic factors of patients with advanced gastric cancer factors.Results There were statistically significant differences in clinical N staging,clinical TNM staging and pathological TNM staging in three groups of patients with advanced gastric cancer(P<0.05).There was no significant difference in the median survival time in three groups of patients with advanced gastric cancer(P>0.05).The 1-year,3-year and 5-year survival rates of group A were higher than those of group B and group C(P>0.05).The factors affecting the prognosis of gastric cancer included the degree of tumor differentiation,pathological T staging,pathological TNM staging,the total number of lymph nodes,the number of positive lymph nodes and lymph node metastasis.Conclusion Combined detection of CEA and CA19-9 expression indicates a certain reference value for prognosis of gastric cancer.

The detection of JAK2 V617F and CALR mutations,which was first reported in 2005 and 2013,respectively,had greatly improved the diagnosis and treatment of myeloproliferative neoplasms (MPN).Most MPN patients are inclined to thrombophilia,the carrier frequency of JAK2 V617F mutation was also high in patients of various thrombotic events,and the relationship between the two conditions needs further study.The observation of JAK2 V617F mutation in control group and general population also enlightened and changed the understanding about the development of chronic cancer.Research progress in this area will be introduced together with the related reports in the 56th American Society of Hematology annual meeting in 2014.