14 Hematopoietic System

14 Hematopoietic System

2015107 Analysis of clinical features and risk factors for the development of hemorrhagic cystitis post allogeneic hematopoietic stem cell transp lantation.YIN Yuming（殷宇明），et al.Beijing Aerospace Center Hosp，Beijing 100049.Chin J Clin Hematol 2014；27（11）：970-972.

ObjectiveTo analyze the clinical features and risk factors of hemorrhagic cystitis（HC）after allogeneic hematopoietic stem cell transplantation（allo-HSCT）.M ethodsClinical data，virological results，and association between HC and GVHD or other factors were analyzed in 150 patients undergoing allo-HSCT.Analysis of variance and multiple linear regressions were used.Resu ltsHC occurred in 32 of the 150 patients with an incidence of 21.3%.The median time to diagnosis of HC was 29 days after allo-HSCT.All cases were late-onset HC. There was no statistic difference between age，gender，diagnosis，morbid state，sex conformity between recipient and donor，conditioning regimen（with or without TBI），type of transplant（with or without the third kind of stem cell），number of MNC and CD34+cell，the time of engraftment for WBC and PLT，EBV viremia and the occurrence of HC.Univariate analysis indicated that acute GVHD，HLA disparity between recipient and donor，ATG usage to prevent GVHD and CMV viremia were associated with HC（P＜0.01）.Multiple regression analysis showed only acute GVHD was the independent risk factor associated with HC.ConclusionAcute GVHD is an independent risk factor of late-onset HC，and there is an association between acute GVHD and late-onset HC.

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2015108 C linical observation of autologous cytokine-induced killer cells for the maintenance treatment of elderly patients w ith multiple myeloma.ZHANG Fan（张凡），et al.Dept Hematol，Benq Med Center，Nanjing 210019.Chin JClin Hematol 2014；27（11）：958-961.

ObjectiveTo evaluate the effectiveness and safety of autologous cytokie induced killer（CIK）cells in maintenance treatment of elderly patientswithmultiplemyeloma（MM）.MethodsFive elderly MM patients with CR orPR were enrolled.Peripheral blood mononuclear cells were isolated and augmented by priming with IFN-γfollowed by IL-2 and monoclonal antibody against CD3. The autologous CIK cells were fractionally transfused back to individual patients and the total amount was（1 to 3）×1010，onemonth as one cycle.First three cycles were given continuously，then once every three months. The condition of remission and quality of life（QOL）were observed.ResultsAfter 9 to 23 months follow-up，32 cycles of CIK cell treatmentwere performed for 5 patients and adverse reaction was mild.The percentages of CD3+，CD3+CD8+and CD3+CD56+increased significantly（P＜0.05）.CIK cell immunotherapy could climinate residual tumor cells and maintain remission status well.QOL was improved in all patients（P＜0.05）.ConclusionAutologous CIK cells is a safe and efficacious maintenance treatment for the 5 elderly patients with MM.

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2015109 Clinical characteristics and prognostic analysis of patients w ith ad renal lym phom a.WU Yi（吴艺），et al.Dept Hematol，Ruijin Hosp，Shanghai Jiaotong Univ Med Sch，Shanghai 200025.Chin JClin Hematol2014；27（11）：947-949.

Ob jectiveTo analyze the clinical characteristics and survival of patients with adrenal lymphoma.M ethodsClinical datas of18 adrenal lymphoma patients treated in our hospital from January 2002 to December 2013 were collected and analyzed.Among them，10 caseswere primary adrenal lymphoma（PAL）and 8 cases were secondary adrenal lymphoma（SAL）.The date of last follow-up wasMarch 30，2014.Survival functions were estimated using the Kaplan-Meier method and compared by the Log-rank test.Resu ltsBoth in PAL and SAL，the main subtype of the adrenal lymphoma was diffuse large B-cell lymphoma，followed by peripheral T-cell lymphoma-not otherwise specified.The major symptoms，in both PAL and SAL，were B symptom，fatigue，regional pain，and anorexia.Hepatosplenomegaly and advanced stage were more frequent in SAL than in PAL（P=0.019 and P= 0.036，respectively）.Considering the patients'overall survival（OS），there was no statistical difference between PAL and SAL.But patients with IPI score≥3 or T-cell derived lymphoma had shorter OS（P=0.049 5 and P= 0.003 4，respectively）.ConclusionThe most common subtype of adrenal lymphoma is diffuse large B-cell lymphoma and patients lack specific symptoms.Patientswith IPI score≥3 or T-cell derived lymphoma have poor outcome.Because of the limited number of patients，the prospectivemulti-center clinical analysis is warranted.

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2015110 Analysis of clinical data in 24 young patientsw ith m ultip lem yeloma.HU Ying（胡影），etal. Dept Hematol，Beijing Chaoyang Hosp Capital Med Univ，Beijing 100043.Chin J Clin Hematol 2014；27（11）：955-957.

Ob jectiveTo explore the clinical features，laboratory results，therapeutic effects，survivals of young patients（age＜50）with multiplemyeloma（MM）.MethodsWe retrospcctively analyzed the clinical data of 24 young patients with MM.Resu ltsYoung patients accounted for 10.1%ofallpatientswith MM.IgG type and lightchain type were the most common.All patients were in DS stagingⅢ，6 patientswere in ISSstagingⅠ，14 patients in ISS stagingⅡand 4 patients in ISS stagingⅢ.Eight patientswere accompanied with extramedullary infiltrate. Cytogenetic abnormalitieswere seen in six patients.Fifteen patients accepted velcade，5 patients accepted lenalidomide，6 patients attended clinical trial.2 patients took liposomal doxorubicin and 2 patients took arsenic trioxide.Allogeneic hematopoietic stem cell transplantation was used in one patient.ORR was91.7%，CR rate was 41.7%.The median time to CR was 6 months and themedian maintaining time was 18 months.One-year survival rate was 100%，three years expecting survival rate was 95.45%，five years expecting survival rate was 70.67%.ConclusionElderly patientsare predisposed to MM，but incidence of the disease is on the rise in young patients.Serious bone damage and extramedullary infiltrate can be often seen in young patients.Treatment effect of velcade and lenalidomide are good.Survive time of young patients is longer than old patients.

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2015111 Study on the im pact of Trp170Ser mutation on the binding mechanism of rFⅧlight chainwith VWF.CHI Kun（迟昆），et al.Dept Lab Med，Ruijin Hosp，Shanghai Jiaotong Univ Med Sch，Shanghai 200025.Chin JHematol 2014；35（11）：995-999.

ObjectiveTo disclose the impact of Trp1707Ser mutation on the binding mechanism of rFⅧlight chain（cFⅧLC）with VWF.M ethodsUsing long-chain PCR technique，we constructed rFⅧLC plasmidsof both wild type and Trp1707Sermutant type.Bl21 competent cells were used for protein expression.Gradient renaturation was employed to refold protein.SDS-PAGE and Western blot were performed to identify the molecular weight of expressed protein.GST-Sefinose was used for protein purification and surface plasmon resonance（SPR）was employed to detect binding of B-domain-deleted rFⅧ（BDD-rFⅧ），wild and mutant rFⅧLC with VWF，respectively.ResultsThe results of SDS-PAGE and Western blot showed a molecular weight of 110×103of expressed proteins，which were consistent with objective proteins.The expression quantity ofwild type was higher than that of mutant type.A concentration-dependent combination of the 3 testing proteins with VWF was found.The KDvalue of BDDrFⅧ（12.2）was lower than that of both rFⅧLCs（wild type 48.9 and mutant type 46.3），whereas there was no discrepancy between wild rFⅧLC and mutant rFⅧLC.ConclusionTrp 1707Ser mutation does not impact the binding of rFⅧLC expressed by BL21 competent cells with VWF.The heavy chain play amore important role in impacting the binding of Fiwith VWF.

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2015112 The changes of circulating follicular regulatory T cells and follicular T helper cells in children immune thrombocytopenia.CUI Yajie（崔亚杰），et al.Dept Hematol，Zhengzhou Children's Hosp，Zhengzhou 450003.Chin JHematol 2014；35（11）：980-984.

ObjectiveTo investigate the changes and roles of follicular regulatory T cells（Tfr）and follicular T helper cells（Tfh）in the pathogenesis of children immune thrombocytopenia（ITP）.Methods32 untreated ITP patients，aswellas20 healthy controlswere enrolled in this study.The proportion of circulating Tfr and Tfh cells were determined by flow cytometry；real-time PCR was performed to detect the expression of transcription factors and regulatory factors of Bcl-6，c-Maf，Blimp-1 and PD-1 mRNA；ELISA was used to detect plasma concentration of IL-2，IL-6，IL-10 and IL-21.Resu lts①The proportion of Tfh cellswere significantly higher（P＜0.05），while the Tfr cells and the ratio of tfr/Tfh cells in ITP patients were significantly lower than that in health controls（P＜0.05）；②Correlation analysis showed that the Tfr cells and the ratio of Tfr/Tfh were positively correlated with the platelet counts and negatively with the levels of PA-IgG，while the proportion of Tfh cells was positively correlated with the levels of PA-IgG and negatively with the platelet counts in peripheral blood；③Transcription levels of Bcl-6 and c-MafmRNA in CD4+T lymphocytes cells were significantly elevated，the Blimp-1 mRNA in CD4+cells and PD-1 mRNA levels of Treg were lower in ITP patients in comparison with healthy controls；④The higher Plasma concentration of IL-21，and lower concentration of IL-2 were found in ITP patients.Conclusion①The lower proportion of Tfr cells and higher proportion of Tfh cells，as well as the abnormal ratio of Tfr/Tfh mightaccount for the decreased platelet counts to be further involved in the immunological pathogenesis of children ITP；②The changes of plasma cytokines IL-2，IL-21 inmicroenvironment and the over-expression of Bcl-6 mRNA，c-MafmRNA and the lower-expression of Blimp-1 mRNA in CD4+T cells，and over-expression of PD-1 mRNA in Treg cells might be account for the abnormal ratios of Tfr/Tfh cells in ITP patients.

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2015113 Increasing peripheral b lood neutrophils after G-CSF treatment is a predictor of early response to immunosuppressive therapy in severe aplastic anem ia.WU Zhijie（武志洁），et al.Instit Hematol& Blood Dis Hosp，CAMS&PUMC，Tianjin 300020.Chin JHematol 2014；35（11）：974-979.

ObjectiveTo testify whether obsolute neutrophil count（ANC）response to preimmunosuppressive-therapy（pre-IST）granulocyte-stimulating factor（G-CSF）treatment could predict early response to IST in severe aplastic anemia（SAA）.MethodsClinical data and hematologic response of 125 SAA patients treated with antithymocyte globulin（r-ATG）combined with cyclosporine were retrospectively analyzed.Correlation of ANC re-sponse to pre-ISTG-CSF treatment and early response to IST were statistically analyzed，and receiver operating characteristic（ROC）curve was used to estimate the value of increased ANC（ΔANC）in predicting early IST response.ResultsThe hematologic response（HR）rate to IST in ANC reponded patients was significantly higher than non-responded group（3-month HR 49.0%vs 28.9%，P=0.023；6-month HR 61.2%vs 40.8%，P=0.026）.WithΔANC≥0.5×109/L as the cutoff level，the best point to predict early IST response was10 days after G-CSF（d 10）.Response of ANC to pre-IST G-CSF treatmentat d 10 was among the independent factors of predicting 3-month（P=0.004），but not for 6-month response to IST.The overall 5-year survival rate was92.8%and 69.5%in ANC responded and non-responed groups，respectively（P=0.025）.ConclusionResponding to pre-ISTG-CSF treatment reflected the residual bone marrow hematopoiesis，and could act as a convenient and practical predictor to early IST response aswell as long-term survival in SAA.

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2015114 C linical characteristics and prognoses of 167 acute erythroleukem ia patients.CHENGWenxiu（程文秀），et al.Jiangsu Instit Hematol，1st Affil Hosp，Soochow Univ，Suzhou 215006.Chin J Hematol 2014；35（11）：970-973.

ObjectiveTo observe the biological characteristic and the prognoses in patientswith acute erythroleukemia（AEL）.M ethodsThe results of 167 patients with new ly diagnosed AEL，from January 2003 and June 2013 in the First Affiliated Hospital of Soochow University，were reviewed by MICM.ResultsFlow cytometry analysis indicated that CD13（96.1%），CD33（95.1%），CD117（87.4%）and CD34（79.4%）were highly expressed in AEL.56 of148（37.8%）AEL patients had a variety of cytogenetic abnormalities，27 of 148（18.2%）patients were complex karyotype（abnormalities involving 3 or more chromosomes），the abnormalities of chromosomes 3，5，7 and 8 were more frequently involved and the most common one was+8，accounting for 35.7%of all abnormal karyotype，followed by 5q-（17.9%）.Mutation analysis showed CEBPA mutation ratio of AEL patientswas44.0%（11/25），thatof NPM1 as15.4%（4/ 26）.Initial induced remission rate of AEL was 56.6%（30/53），compared with 33.3%（4/12）of MDSM6. Survival analysis showed that the overall survival in female was better than that in male（P=0.047）.The overall survival time of transplantation group is significantly longer than chemotherapy group（P=0.000）. The OS of 13-39 years old group was the best，40-49 years old group took second place，＞50 years old group appeared to be the worst.ConclusionAEL had its own unique biological features，and allogeneic hematopoietic stem cell transplantation could significantly improve its poor prognosis.

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2015115 Prim ary p leural diffuse large B cell lymphoma：a case report and review of literature.SUN Meiling（孙美玲），et al.Dept Pulmon Med.Shandong Provin Hosp Affil Shandong Univ，Jinan 250021.Chin J Tuberc Respir Dis2014；37（11）：835-839.

ObjectiveTo analyze the clinical features of 1 case of primary pleural diffuse large B-cell lymphoma（DLBCL）and therefore to improve the understanding of this disease.M ethodsThe clinical features，auxiliary examinations and diagnosis of a case with DLBCL were reported and the related literatureswere reviewed.The literature review was carried out respectively with“primary pleural，lymphoma”，as the search terms in Wanfang Data，CNKIand PubMed by December2013.ResultsA 73 year-oldmale patientwas admitted to the hospital because of chest pain on the left side for 1 month.The main symptoms of the patient were left side chest pain，with occasional cough.ChestCT scan showed leftpleural effusion and pleural thickening.Thoracoscopy was performed and revealed multiple sizes of nodules on the visceral and parietal pleura.Pathology study confirmed the diagnosis of DLBCL.The patientwas treated with CHOP regimen 3 times.So far，there was no local recurrence. A total of 12 literatureswere retrieved from Wanfang Data，CNKIand PubMed，and they were all case reports. There were 8 male and 4 female patients，aging from 12 to 81 years.Themain symptomswere chest pain and fever.ConclusionPrimary pleural DLBCL is a rare disease.It is easily to be misdiagnosed due to non-specific clinical and imaging manifestations.The final diagnosisdepends ultimately on pathological biopsy，and thoracoscopy is the most effectivemethod to confirm DLBCL.

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2015116 A study of prognostic value of cytogenetics in patients w ith primary m yelofibrosis.LI Bing（李冰），et al.Instit Hematol&Blood Dis Hosp，CAMS& PUMC，Tianjin 300020.Chin JHematol2014；35（11）：990-994.

Ob jectiveTo evaluate the prognostic value of cytogenetics in Chinese with primary myelofibrosis（PMF）.MethodsFour hundred and thirty-nine Chinese patients with PMF were retrospectively analyzed.The Kaplan-Meiermethod，the Log-rank test，the likelihood ratio test and the COX proportional hazards regression model were used to evaluate the prognostic scoring systems.ResultsFour hundred and thirty-nine Chinese patients with PMF were analyzed with amedian age of 56 years（range：8-83），including 298 males and 141 females.The DIPSS-plus system could effectively evaluate prognosis in Chinese patients with PMF.There was significantly higher predictive power for survival for the DIPSS-plus group compared with the DIPSS group（P=0.006，-2 loglikelihood ratios of989.5 and 1001.9 for the DIPSS-plus and DIPSS systems，respectively）.Univariate analysis indicated that the patients with a normal karyotype，a complex karyotype that was not a monosomal karyotype，+8 only or a balanced translocation only had better survival.Following two cytogenetic risk categorieswere constructed：favorable karyotype including subjects with a normal karyotype，a complex karyotype that was not a monosomal karyotype，+8 only or a balanced translocation only and unfavorable karyotype included all others. The modified DIPSS-Chinese prognostic model was proposed by adopting cytogenetic categories and DIPSS-Chinese risk group.The median survival of patients classified in low risk（163 subjects），intermediate-1 risk（187 subjects），intermediate-2 risk（82 subjects）and high risk（7 subjects）were not reached，74（95%CI 42-106），39（95%CI 26-52）and 12（95%CI 1-25）months，respectively，and there was a statistically significant difference in overall survival among the four risk groups（P＜0.01）.ConclusionThe DIPSS-plus had significantly higher predictive power than the DIPSS group in Chinese patients with PMF and the modified DIPSS-Chinese system based on the cytogenetic features of Chinese patients was proposed and worked well for prognostic indication.

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