Objectives: To investigate the clinical and genetic features of young Chinese patients with myeloproliferative neoplasms (MPN). Methods: In this cross-sectional study, anonymous questionnaires were distributed to patients with MPN patients nationwide. The respondents were divided into 3 groups based on their age at diagnosis: young (≤40 years) , middle-aged (41-60 years) , and elderly (>60 years) . We compared the clinical and genetic characteristics of three groups of MPN patients. Results: 1727 assessable questionnaires were collected. There were 453 (26.2%) young respondents with MPNs, including 274 with essential thrombocythemia (ET) , 80 with polycythemia vera (PV) , and 99 with myelofibrosis. Among the young group, 178 (39.3%) were male, and the median age was 31 (18-40) years. In comparison to middle-aged and elderly respondents, young respondents with MPN were more likely to present with a higher proportion of unmarried status (all P<0.001) , a higher education level (all P<0.001) , less comorbidity (ies) , fewer medications (all P<0.001) , and low-risk stratification (all P<0.001) . Younger respondents experienced headache (ET, P<0.001; PV, P=0.007; MF, P=0.001) at diagnosis, had splenomegaly at diagnosis (PV, P<0.001) , and survey (ET, P=0.052; PV, P=0.063) . Younger respondents had fewer thrombotic events at diagnosis (ET, P<0.001; PV, P=0.011) and during the survey (ET, P<0.001; PV, P=0.003) . JAK2 mutations were found in fewer young people (ET, P<0.001; PV, P<0.001; MF, P=0.013) ; however, CALR mutations were found in more young people (ET, P<0.001; MF, P=0.015) . Furthermore, mutations in non-driver genes (ET, P=0.042; PV, P=0.043; MF, P=0.004) and high-molecular risk mutations (ET, P=0.024; PV, P=0.023; MF, P=0.001) were found in fewer young respondents. Conclusion: Compared with middle-aged and elderly patients, young patients with MPN had unique clinical and genetic characteristics.

Objectives: To explore health-related quality of life (HRQoL) and identify its associated variables in Chinese patients with Philadelphia-negative myeloproliferative neoplasms (MPNs) . Methods: In this cross-sectional study, anonymous questionnaires were distributed to adult patients with MPNs to assess symptom burden measured by MPN-10 and HRQoL measured by Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36) and the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire Core 30 (EORTC QLQ-C30) . Results: The data from 1405 respondents with MPNs, including 645 (45.9%) with essential thrombocythemia (ET) , 297 (21.1%) with polycythemia vera (PV) , and 463 (33.0%) with myelofibrosis (MF) , were analyzed. 646 (46.0%) respondents were male. The median age was 56 (range, 18-99) years. The mean MPN-10 scores were 13.0±12.7, 15.0±14.7, and 21.0±16.6 (P<0.001) , and the physical component summary (PCS) and mental component summary (MCS) scores were 48.0±8.5, 47.0±9.0, and 42.0±10.0 (P<0.001) and 51.0±11.0, 50.0±10.8, and 49.0±11.1 (P=0.002) for respondents with ET, PV, and MF, respectively. Respondents with MF reported the lowest score of physical functioning, role functioning, emotional functioning, cognitive functioning, social function, and global health status (all P<0.01) and the highest score of fatigue, pain, dyspnea, appetite loss, diarrhea, and financial problems (all P<0.05) in EORTC QLQ-C30. Multivariate analyses revealed that higher MPN-10 scores were significantly associated with lower PCS (-0.220 to -0.277, P<0.001) and MCS (-0.244 to -0.329, P<0.001) scores; increasing age (-1.923 to -4.869; all P<0.05) , lower PCS score. Additionally, comorbidity (ies) , symptom at diagnosis, splenomegaly, anemia, unknown driver gene, and higher annual out-of-pocket cost were significantly associated with lower PCS and/or MCS scores. However, age ≥ 60 years, urban household registration, concomitant medication, and receiving ruxolitinib therapy in respondents with MF were associated with higher MCS scores. Weak correlations were found between MPN-10 score (except the subscale of appetite loss and constipation) and EORTC QLQ-C30 score in majority of subscales in respondents with ET (|r| = 0.193-0.457, all P<0.001) , PV (|r| = 0.192-0.529, all P<0.01) , and MF (|r| = 0.180-0.488, all P<0.001) , respectively. Conclusions: HRQoL in patients with MPN was significantly reduced, especially in patients with MF. Sociodemographic and clinical variables were significantly associated with the HRQoL in patients with MPNs.

Objective: To assess the value of immunoglobulin heavy/light chain (HLC) immunoassay on therapeutic response in patients with multiple myeloma(MM). Methods: A total of 45 newly diagnosed MM patients were retrospectively enrolled in Peking Union Medical College Hospital from 2013 to 2016, whose 115 serum samples were consecutively collected. HLC was tested to evaluate response and compare with other methods for M protein detection. Results: ①There were 30 males and 15 females in total of whom the monoclonal immunoglobulin was IgG in 27 (IgGκ∶IgGλ 12∶15) and IgA (IgAκ∶IgAλ 9∶9) in 18. The arerage age of the studied population was 59 (range 43-80) . ② In 34 patients with serum sample at diagnosis, 32 (94.1%) had abnormal HLC ratio (rHLC) while 2 patients with IgG had normal rHLC. The percentages of abnormal rHLC was 81.8% (18/22) at partial response、50.0%(9/18) at very good complete response and 16.0%(4/25) at complete response. ③In 25 patients reaching CR, there were 13 with IgG and 12 with IgA. 4 patients equally split of IgG and IgA had abnormal rHLC at complete response. ④By monitoring the rHLC of some patients consecutively, we found that the remission of rHLC was to some extent behind the remission of SPE and IEF, or even rFLC. Conclusion: Immunoglobulin HLC detection is one feasible method for minimal residual disease detection.

Objective:Analysis on traditional Chinese medicine syndrome of 220 cases of patients with aplastic anemia(110 severe aplastic anemia and 110 light aplastic anemia)was made,hoping to explore basic syndromes of the aplastic anemia through the comparative analysis,to observe the similarities syndrome characteristics or differences between severe and light aplastic anemia,and to provide theoretical basis of Chinese medicine treatment for aplastic anemia.Methods:The retrospective analysis on the general conditions(sex,age,course of disease,peripheral blood),symptoms,syndromes and factors which may be associated with the syndromes in 220 cases of aplastic anemia patients was made.Conclusion:Deficiency of Qi,deficiency of blood,deficiency of kidney-yang and deficiency of kidney-yin are basic syndromes of aplastic anemia.While the deficiency of Qi and blood is more prominent than deficiency of yin and yang,especially in the severe aplastic anemia.Drugs for supplementing Qi and nourishing blood must be reused in treatment of traditional Chinese medicine.The frequency of intense noxious heat syndrome in severe aplastic anemia was higher than that in light aplastic anemia,and in daily life infection protection for SAA should be taken seriously attention.

Objective To observe the influence of Yiqi Yangxue Huoxue Fang on platelet parameters in the patients with refractory idiopathic thrombocytopenic purpura(RITP).Methods The patients with RITP(n=40) were included into the treatment group treated Yiqi Yangxue Huoxue Fang,and other health people(n=36),into the control group.The platelet count(PLT),palteletcrit(PCT),mean platelet vojume(MVP) and platelet distribution width(PDW) in treatment group were compared with those in control group before the treatment,and were compared and observed in treatment group before and after the treatment.Results Before the treatment,PLT(P<0.01) and PCT(P<0.05) were lower and MPV and PDW were higher(P<0.05) in treatment group than those in control group.PLT and PCT increased significantly(P<0.01,P<0.05),and MPV and PDW decreased significantly(P<0.05) in treatment group after the treatment compared with those before the treatment.Conclusion Yiqi Yangxue Huoxue Fang can influence the platelet parameters like PCT,MPV and PDW besides increasing PLT in RITP patients

Objective To analyse the basic TCM syndromes of immunologic thrombocytopenic purpura (ITP). Methods The hospitalization materials were retrospectively summarized in 149 patients with ITP and 49 ones with secondary thrombocytopenia, and the clinical symptoms and syndromes were analysed statistically. Results The cases of ITP onset were more in female patients than in male ones. The first three syndromes with higher occurrence frequency were, in proper order, qi deficiency (85.4%), blood stasis (44.4%) and blood deficiency (42.4%). Conclusion The main TCM syndromes of ITP include qi deficiency, blood stasis and blood deficiency. The bleeding mechanism of ITP is related to blood failing to circulate in the vessels induced by qi failing to control blood and internal stagnation of blood stasis.

Objective:To study the treatment of Acute Myeloid Leukemia by Integration of Traditional Chinese and Western medicine during remission induction.Methods:With retrospective analysis,this paper summaries the clinical effect and does comparison research on 20 new AML patients.The patients are divided into two groups according to whether taking traditional Chinese medicine,Chinese medicine and chemotherapy group(treatment group) and chemotherapy-only group(control group).This paper observes clinical treatment effect of AML patients during remission induction.By observing clinical effect(complete remission rate,effective rate and improvement of Chinese medicine syndrome),supportive treatment,chemotherapy side effects,the paper analyses whether traditional Chinese medicine has its own advantages.Results:(1) CR rate and effective rate of two groups are 50% and 60%,60% and 70% respectively.Both CR rate and effective rate of treatment group is lower than the control group,but with no significant difference.(P0.05).(2)Red blood cell transfusions for the treated group are significantly less than the control group(P0.05) during chemotherapy.Platelet transfusion and usage of Granulocyte Colony-stimulating factor for the treated group are less than the control group with no significant difference(P0.05).(3)The comparison of side-effects during chemotherapy:The liver damage incidence of the treated group is pretty lower than the one of control group,30% and 80% respectively.Two sets of data have significant difference(P0.05).Conclusion:(1) for treatment of AML,traditional Chinese medicine shows considerable advantages in supportive treatment,because it can reduce blood transfusions.(2)Traditional Chinese medicine has begun to deliver results in reducing the toxic and side effect of the chemotherapy drugs.(3) Is involvement of traditional Chinese medicine good to increasing CR rate of AML patients? This is still debatable.

目的:观察中西医联合治疗系统性红斑狼疮的疗效.方法:采用益气养血补肾法联合大剂量环磷酰胺(CTX)冲击治疗SLE30例,记录治疗前后T细胞亚群(CD+4,CD+8,CD+4/ CD+8)及NK细胞等指标并观察疗效.结果:治疗总有效率90%;并且70%活动期SLE患者出现CD+4,降低、CD+4/CD+8倒置、CD+8升高趋势,且随着病情的好转CD4+、CD+4/ CD+8比值回升(P＜0.05或＜0.01),CD+8降低(P＞0.05);同样,大部分(77%)活动期SLE患者NK细胞数也随着病情好转而回升(P＜0.05).结论:益气养血补肾法可改善T细胞亚群、NK细胞的失衡,提高疗效.

Objective To investigate the influence of p53 mutation status in tumor cells to the gene therapy of Adv-p53.Methods The mutation status of p53 gene in MCA-205 and Panc-02 cells was analyzed by SSCP-PCR and DNA sequence.The transduction ratio of adenovirus vectors was analyzed by flow cytometric analysis.Apoptosis of tumor cells transfected with Adv-p53 was analyzed by TUNEL assay.Anti-tumor effect of Adv-p53 was examined in murine liver metastasis model.Results p53 gene in MCA-205 cells had a mutation in 769th base from G to T.p53 gene in Panc-02 cells had not a mutation.The transduction ratio of adenovirus vectors was higher in that of Panc-02 cells,but apoptosis after transfected with Adv-p53 was more significant in MCA-205 cells.In vitro experiment,growth of tumor cells was inhibited by Adv-p53 in MCA-205 cells,but not in Panc-02 cells.In murine liver metastasis model,the growth of MCA-205 tumors was inhibited by intra-portal administration of Adv-p53,but the growth of Panc-02 tumors was not inhibited.Conclusion The anti-tumor effect of Adv-p53 was more significant in tumor cells with a mutation of p53 gene.The effect of gene therapy of Adv-p53 was influenced by p53 mutation status in tumor cells

Objective To observe the curative effect of the therapy of replenishing qi, nourishing blood and tonifying kidney on systemic lupus erythematosus (SLE).Methods The therapy of replenishing qi, nourishing blood and tonifying kidney combined with cyclophosphamide (CTX) impact therapy was used to treat SLE in 30 cases.The curative effect, times of CTX impacts when the patients get obvious effects or effective results, changes of laboratory indexes before and after treatment, hormone minimum maintenance dose, toxic and side effects were observed, and compared with relevant documents both at home and abroad.Results The total effective rate was 90% in 30 cases after the treatment.The times of CTX impacts were different as SLE involved systems were different.In 22 cases the positive antigen of ds-DNA conversed negatively after the treatment, erythrocyte sedimentation rate (ESR) decreased significantly (P0.01), levels of complement3 (C3) and complement4 (C4) increased significantly (P0.05), and immunoglobulin G (IgG) decreased significantly (P 0.05).The hormone minimum maintenance dose was lower and toxic and side effects were less.Conclusion The therapy of replenishing qi, nourishing blood and tonifying kidney combined with CTX impact therapy has good curative effect on SLE.

[Purpose] To evaluate the value of interleukine-6(IL-6),C-reaction protein(CRP),β2-microglobulin(β2-MG) and lactatede hydrogenase(LDH) in efficacy and prognosis of multiple myeloma(MM) patients.[Methods] One handred and ten laboratory data from seventeen stage Ⅲ cases teated with Chinese Medicine and chemotherapy were analyzed for correlation.Eight cases were compared before and after treatment.[Results] Levels of IL-6,CRP,CRE,Urea,β2-MG,BMPC and Ben Jones protein significantly reduced after treatment(P0.05).Correlations were found between IL-6 and CRP,β2-MG and LDH,β2-MG and Urea,β2-MG and BMPC,LDH and Ben Jones protein(P0.01,|r|0.45),IL-6 and Ca(P0.05,|r|0.45).β2-MG levels in 3A and 3B patients were significantly different(P0.01,|r|0.45),and their correlations with BMPC were both significant(P0.05,|r|0.45).[Conclusion] Levels of IL-6,CRP and β2-MG are suitable efficacy and prognostic factors in stage Ⅲ multiple myeloma cases.

目的 探索高效的大鼠胰岛分离移植技术方法.方法 应用Wistai-Furth大鼠,于体内或体外胶原酶经胰管灌注膨化胰腺,联合不同密度Ficoll液或Histopaque液纯化胰岛细胞,评估胰岛的数量、纯度、胰岛当量以及肾被膜下胰岛移植的有效性.结果 体外经胰管灌注膨化胰腺结合Histopaque液纯化提取胰岛的数量、纯度和胰岛当量值均显著高于体内灌注组各数值(P＜0.01),其提取时间无显著差别.1000个胰岛细胞移植进入左肾被膜下,有效的逆转了糖尿病大鼠高血糖,其远期糖耐受结果优于500和800个胰岛细胞移植组.结论 体外灌注膨化消化胰腺结合Histopaque液纯化胰岛的分离方法是一种满意的分离技术.1000个胰岛细胞是保证肾被膜下胰岛移植成功的最低有效数量.

Objective The sensitivity of tumor cells to interferon-γ(IFN-γ) was investigated us- ing two kinds of tumor cells separated from Panc-02 cells,whose sensitivities to IFN-γwere different. Methods After Panc-02 cells were stimulated with IFN-γ,FS-3 and PM-9 cells were separated based on the expression of MHC I by flow eytometry ceil-sorting techniques.These tumor cells were injected sub- cutaneously in mice.The sizes of subcutaneous tumors were monitored in 21 days.Results After FS-3, Pane-02 and PM-9 cells were stimulated with IFN-γ,the expression of MHC I was different.FS-3 cells were sensitive to IFN-γ,and PM-9 cells insensitive to IFN-γ.After these tumor cells were injected subcu- taneously in mice,the growth of PM-9 tumors was faster than that of FS-3 and Pane-02 tumors.After 21 days,the sizes of PM-9 tumors (139.1±30.2) mm~2 were larger than those of FS-3 and Pane-02 tumors [(0.8±1.6),(14.3±6.7) mm~2,P＜0.01].On the 21st day,PM-9 tumors were observed in all of mice (n=24,10096),FS-3 tumors and Pane-2 tumors were observed in parts of mice (n=12,30 % and n=24,75 % respectively;P＜0.05).Conclusion The sensitivity of tumors to IFN-γaffects its tumori- genicity in in vivo experiment.

Objective To discuss the principals and methods of surgical treatment for advanced age patients with colon cancer complicated with acute obstruction.Methods Seventy nine advanced age patients with colon cancer complicated with acute obstruction diagnosed from 1993 to 2002 were analyzed retrospectively.Seventy eight of them were treated by operation.Results Forty seven patients received one-stage operation,including 11 radical excision,36 wedge resection;23 Hartmann operation;5 colostomy;two ileocolic anastomosis and one exploratory laparotomy.Sixty one patients were with well-differentiated or moderately differentiated adenocarcinoma,10 with poorly differentiated adenocarcinoma and 8 were with mucinous adenocarcinoma.Conclusion The choice of operation for the advanced age patients with colon cancer complicated with acute obstruction is dependent on the specific condition of the patients.There are several choices of operation,such as on-stage operation and staging operation.On the basis of the characteristic of advanced age patients,the perioperative management should be emphasized.

目的观察益气养血补肾法治疗重型再生障碍性贫血的临床疗效.方法用益气养血补肾法合康力龙治疗重型再生障碍性贫血33例,观察治疗后患者临床表现、血常规等指标的变化.结果治疗后证候学积分较疗前有显著性下降(P<0.01),在改善症状方面有效率为90.91%,临床疗效总有效率88.24%,患者的血色素从疗后第6个月开始较疗前升高(P<0.01),白细胞计数比疗前升高(P＜0.05),疗后网织红细胞计数较疗前有显著性升高(P<0.01).结论益气养血补肾法合康力龙对重型再生障碍性贫血患者有较好的临床疗效.

研究目的:研究中药浙贝母逆转白血病(肿瘤)细胞多药耐药的作用.研究内容:①临床研究:将90例急性白细胞患者分为治疗组(48例,在常规化疗方案的基础上同时加用浙贝母粉口服)及对照组(42例,选用常规化疗方案),两组在正规化疗疗程后,按缓解情况统计临床疗效.结果:在临床安全剂量下,浙贝母粉与常规化疗方案合用,可以明显降低白血病患者骨髓细胞耐药蛋白p-糖蛋白的表达;降低骨髓中白血病细胞百分比;明显提高急性白血病患者,尤其耐药蛋白p-糖蛋白高表达的病例和难治、复发病例的完全缓解率.②实验研究:从浙贝母总生物碱中分离得到的贝母碱甲素、贝母碱乙素是主要活性成分,属于异甾类生物碱中的瑟闻琴类生物碱.是通过直接抑制p-糖蛋白的表达,减少抗癌药物的"泵"出,增加抗癌药物在耐药肿瘤细胞内的蓄积的.③作为新药开发的前期研究:对浙贝母总生物碱进行了药效、毒理、药理以及药代动力学的探索性研究.

Objective To investigate the anti tumor effect of IL 12 administration into the portal system in treatment of the liver metastasis in mice. Methods 1×10 5 of MCA 205 were injected into the portal vein to establish murine liver metastasis model, and the spleen was transpositioned subcutaneously for administration of IL 12 continually into the portal system. From day 3 to 7, 0 1 μg IL 12 was administered intraperitoneally or intra splenicly, while HBSS was injected intra splenicly in the control group. Tumors in the liver were examined on day 20. Results The liver weight in the IL 12 intraperitoneal treatment group (1 33±0 08 g) and in the IL 12 intra splenic treatment group (1 29±0 07 g) was significantly less than those in the HBSS group (1 92±0 17 g, P0 05). The number of metastatic nodules in the IL 12 intraperitoneal treatment group (1 53±0 58) and in the IL 12 intra splenic treatment group (0 60±0 89) was significantly less than that in the HBSS group (18 25±5 71, P0 05). Complete regression of the tumor was observed in three of six mice of IL 12 intra splenic treatment group. Conclusion Local administration of IL 12 into the portal system would be a useful strategy for the liver metastasis treatment.

原发性胃肠道淋巴瘤(primary gastrointestinal lymphoma, PGL)的定义是具有明显胃肠道症状或病变,同时组织学证明病变为原发于胃肠道的淋巴瘤[1].Isaacson 和 Wright 提出粘膜相关淋巴组织(mucosa-associated lymphoid tissue, MALT)的概念[2],即与粘膜相关的淋巴组织可以发生淋巴瘤.胃肠道是结外与粘膜相关的最大淋巴器官,MALT淋巴瘤多见于Peyers结、阑尾、支气管粘膜.这一新的淋巴瘤组织学分型已经被广泛接受,从而使我们对于PGL的定义有了新的认识.

目的初步探讨浙贝母散剂和化疗合用时是否能起到逆转P170高表达的作用及其临床应用前景.方法.应用流式细胞仪检测急性白血病患者P糖蛋白(P170)表达,并对阳性患者随机分组,用浙贝母散剂加联合化疗进行逆转多药耐药的临床研究.结果.浙贝母散剂在临床安全剂量下,与常规化疗方案合用,仍可逆转P170高表达;浙贝母散剂与急性白血病(AL)常规化疗方案合用可以降低骨髓原始细胞百分比,有可能提高临床治疗完全缓解率.结论.浙贝母散剂可能成为一种安全有效的多药耐药逆转剂,在P170高表达的急性白血病中有较大的临床意义及推广价值.