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Chinese Journal of Cancer Research 2017年第2期29卷 156-165页

作者： Heng Li Wenjie Xiong Huimin Liu Shuhua Yi Zhen Yu Wei Liu Rui Lyu Tingyu Wang Dehui Zou Zengjun Li Lugui Qiu State Key Laboratory of Experimental Hematology Institute of Hematology and Blood Disease Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Tianjin 300020 China

Objective： This study aims to evaluate the natural history of patients with chronic lymphocytic leukemia （CLL） and a 17p deletion （17p-） and identify the predictive factors within this subgroup. Methods： The sample of patients with CLL were analyzed by fluorescence in situ hybridization for deletions in chromosome bands 1 lq22, 13q14 and 17p13; trisomy of bands 12q13; and translocation involving band 14q32. The data from 456 patients with or without a 17p- were retrospectively collected and analyzed. Results： The overall response rate （ORR） in patients with a 17p- was 56.9%, and patients with a high percentage of 17p- （defined as more than 25% of cells harbouring a 17p-） had a lower ORR. The median overall survival （OS） in patients with a 17p- was 78.0 months, which was significantly shorter than the OS in patients without this genetic abnormality （median 162.0 months, P〈0.001）. Within the subgroup with a 17p-, the progression-free survival was significantly shorter in patients at Binet stage B-C and patients with elevated lactate dehydrogenase （LDH）, B symptoms, unmutated IGHVand a high percentage of 17p-. Conclusions： These results indicated that patients with a 17p- CLL have a variable prognosis that might be predicted using simple clinical and laboratory characteristics.

关键词： 17p deletion chronic lymphocytic leukemia （CLL） fluorescent in situ hybridization （FISH） del 17pin CLL

The eighth International Forum on Stem Cells: virtual meeting, October 20–21, 2022

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Blood Science 2023年第1期5卷 66-72页

作者： Brian Eyden Xiaochen Wang The Christie NHS Foundation Trust Manchester M204BXUnited Kingdom State Key Laboratory of Experimental Hematology Institute of Hematology&Blood Diseases HospitalChinese Academy of Medical SciencesTianjinChina

Cancer accounts for significant levels of human morbidity and mortality.Lymphoma and leukemia are representative of the blood cancers.This dictates the moral imperative for fundamental research leading to clinical applications and therefore better outcomes,but also for conferences of transnational scope,such as the International Forum on Stem Cells,to share knowledge and experience.

关键词： mortality Cancer clinical

Reduced ABO blood group antibody titers in patients after CD19 CAR-T cell therapy

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Blood Science 2023年第1期5卷 62-65页

作者： Qiang Li Zhihuan Yang Kuo Fang Shuning Wei Jiali Sun Wei Liu Xiaojuan Chen Wenyang Huang Guangji Zhang Yin Shi Yuntao Liu Xiaoyuan Gong Fang Liu Xueli Zhou Jianxiang Wang Ying Wang State Key Laboratory of Experimental Hematology National Clinical Research Center for Blood DiseasesHaihe Laboratory of Cell EcosystemTianjin Key Laboratory of Cell Therapy for Blood DiseasesInstitute of Hematology&Blood Diseases HospitalChinese Academy of Medical Sciences&Peking Union Medical CollegeTianjin 300020China

1.INTRODUCTION With rapid developments in genetic engineering,tumor immunology,and cellular engineering,chimeric antigen receptor T cell(CAR-T)cell therapy has become a novel immunotherapy for oncology and other medical fields.1 The promising results of CD19 CAR-T treating B-cell malignancies were reported.2,3 Simultaneously,there existed many adverse events,the most reported of which including B-cell aplasia,hematological toxicity,cytokine release syndrome(CRS),and immune effector-cell–associated neurotoxicity syndrome(ICANS),3,4 but there is still lack of reports demonstrating the impact of CD19 CAR-T on the ABO blood group potency of patient’s serum.

关键词： ABO CD19 CAR

The novel SLC40A1(T419I)variant results in a loss-of-function phenotype and may provide insights into the mechanism of large granular lymphocytic leukemia and pure red cell aplasia

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Blood Science 2022年第1期4卷 29-37页

作者： Hongfei Wu Xiang Ren Meili Ge Peiyuan Dong Shichong Wang Huiming Yi Xingxin Li Jiali Huo Xuan Zheng Mengying Gao Jinbo Huang Jing Zhang Min Wang Peng Jin Neng Nie Yingqi Shao Yizhou Zheng State Key Laboratory of Experimental Hematology National Clinical Research Center for Blood DiseasesInstitute of Hematology&Blood Diseases HospitalChinese Academy of Medical Sciences&Peking Union Medical CollegeTianjin 300020China

Variants in the solute carrier family 40 member 1(SLC40A1)gene are the molecular basis of ferroportin disease,which is an autosomal dominant hereditary hemochromatosis.Here,we present a patient with pure red cell aplasia(PRCA)and large granular lymphocytic leukemia(LGLL)associated with an extremely high levels of serum ferritin and iron overload syndrome.Whole exon sequencing revealed a novel heterozygous variant in SLC40A1(p.T419I),which was found in his daughter as well.A series of functional studies in vitro of the T419I variant in ferroportin were conducted and the results revealed a reduced capacity of iron export from cells without changes in protein localization and its sensitivity to hepcidin.Intracellular iron storage in mutated cells was significantly higher than that of wild-type.These findings suggest that the novel variant p.T419I can cause the classical form of ferroportin disease and an elevated intracellular iron level indicates a potential novel pathogenic mechanism underlying PRCA and LGLL.

关键词： Ferroportin disease Iron overload Large granular lymphocytic leukemia Pure red cell aplasia SLC40A1 variant

Regulatory mechanisms of retinal ganglion cell death in normal tension glaucoma and potential therapies

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Neural Regeneration Research 2023年第1期18卷 87-93页

作者： Wen-Cui Shen Bing-Qing Huang Jin Yang Tianjin Eye Hospital&Eye Institute Tianjin Key Laboratory of Ophthalmology and Visual ScienceNankai UniversityTianjinChina State Key Laboratory of Experimental Hematology National Clinical Research Center for Blood DiseasesInstitute of Hematology&Blood Diseases HospitalChinese Academy of Medical Sciences&Peking Union Medical CollegeTianjinChina

Normal tension glaucoma(NTG)is a multifactorial optic neuropathy characterized by normal intraocular pressure,progressive retinal ganglion cell(RGC)death,and glaucomatous visual field loss.Recent studies have described the mechanisms underlying the pathogenesis of NTG.In addition to controlling intraocular pressure,neuroprotection and reduction of RGC degeneration may be beneficial therapies for NTG.In this review,we summarized the main regulatory mechanisms of RGC death in NTG,including autophagy,glutamate neurotoxicity,oxidative stress,neuroinflammation,immunity,and vasoconstriction.Autophagy can be induced by retinal hypoxia and axonal damage.In this process,ischemia can cause mutations of optineurin and activate the nuclear factor-kappa B pathway.Glutamate neurotoxicity is induced by the over-stimulation of N-methyl-D-aspartate membrane receptors by glutamate,which occurs in RGCs and induces progressive glaucomatous optic neuropathy.Oxidative stress also participates in NTG-related glaucomatous optic neuropathy.It impairs the mitochondrial and DNA function of RGCs through the apoptosis signal-regulating kinase-JUN N-terminal kinase pathway.Moreover,it increases inflammation and the immune response of RGCs.Endothelin 1 causes endothelial dysfunction and impairment of ocular blood flow,promoting vasospasm and glaucomatous optic neuropathy,as a result of NTG.In conclusion,we discussed research progress on potential options for the protection of RGCs,including TANK binding kinase 1 inhibitors regulating autophagy,N-methyl-D-aspartate receptor antagonists inhibiting glutamate toxicity,ASK1 inhibitors regulating mitochondrial function,and antioxidants inhibiting oxidative stress.In NTG,RGC death is regulated by a network of mechanisms,while various potential targets protect RGCs.Collectively,these findings provide insight into the pathogenesis of NTG and potential therapeutic strategies.

关键词： autophagy endothelin 1 glutamate neurotoxicity inhibitor nerve regeneration neuroinflammation normal tension glaucoma oxidative stress retinal ganglion cell vasoconstriction

Chronic Lymphocytic Leukemia Prognostic Index： A New Inteorated Scorino System to Predict the Time to First Treatment in Chinese Patients with Chronic Lymphocytic Leukemia

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Chinese Medical Journal 2017年第2期130卷 135-142页

作者： Heng Li Shu-Hua Yi Wen-Jie Xiong Hui-Min Liu Rui Lyu Ting-Yu Wang Wei Liu Shi-Zhen Zhong Zhen Yu De-Hui Zou Yan Xu Gang An Zeng-Jun Li Lu-Gui Qiu Department of Lymphoma and Myeloma State Key Laboratory of Experimental Hematology Institute of Hematology and Blood Disease Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Tianjin 300041 China

Background： The established clinical staging systems （Rai/Binet） of chronic lymphocytic leukemia （CLL） cannot accurately predict the appropriate treatment of patients in the earlier stages. In the past two decades, several prognostic factors have been identified to predict the outcome of patients with CLL, but only a few studies investigated more markers together, To predict the time to first treatment （TTFT） in patients of early stages, we evaluated the prognostic role of conventional markers as well as cytogenetic abnormalities and combined them together in a new prognostic scoring system, the CLL prognostic index （CLL-PI）. Methods： Taking advantage of a population of 406 untreated Chinese patients with CLL at early and advanced stage of disease, we identified the strongest prognostic markers of TTFT and, subsequently, in a cohort of 173 patients who had complete data for all 3 variables, we integrated the data of traditional staging system, cytogenetic aberrations, and mutational status of immunoglobulin heavy chain variable region （1GI：tV） in CLL-PI. The median follow-up time was 45 months and the end point was TTFT. Results： The median TTFT was 38 months and the 5-year overall survival was 80%. According to univariate analysis, patients of advanced Rai stages （P 〈 0.001） or with 11q- （P = 0.002）, 17p- （P 〈 0.001）, unmutated IGHV （P 〈 0.001）, negative 13q- （P = 0.007） and elevated lactate dehydrogenase levels （P = 0.001 ） tended to have a significantly shorter TTFT. And subsequently, based on multivariate Cox regression analysis, three independent factors for TTFT were identified： advanced clinical stage （P = 0.002）, 17p- （P = 0.050） and unmutated 1GHV （P = 0.049）. Applying weighted grading of these independent factors, a CLL-PI was constructed based on regression parameters, which could categorize tbur different risk groups （low risk [score 0], intermediate low [score 1], intermediate high [score 2] and high risk [score 3-6]） with significantly different TTFT （median TTFT of not reached （NR）, 65.0 months, 36

关键词： 17p Deletion Chronic Lymphocytic Leukemia Immunoglobulin Heavy Chain Variable Mutation Prognostic Index Timeto First Treatment

Mature plasmacytoid dendritic cells associated with acute myeloid leukemia show similar genetic mutations and expression profiles to leukemia cells

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Blood Science 2022年第1期4卷 38-43页

作者： Xiaoyuan Gong Chunhong Li Ying Wang Qing Rao Yingchang Mi Min Wang Hui Wei Jianxiang Wang Leukaemia Center State Key Laboratory of Experimental Hematology National Clinical Research Center for Blood Diseases Institute of Hematology&Blood Diseases HospitalChinese Academy of Medical Sciences&Peking Union Medical College Tianjin 300020China

Introduction:Mature plasmacytoid dendritic cells(pDCs)proliferation associated with myeloid neoplasms(MPDMN)are recognized as a neoplasm related to fully differentiated pDCs.Although it has been reported for many years,the genomic landscape of MPDMN is poorly understood.Methods:We reported two patients who developed acute myeloid leukemia(French-American-British M5 subtype)coexisted with immunophenotypically mature pDCs proliferation,which fit the diagnosis of MPDMN.We sorted pDCs from myeloid blasts by flow cytometry and performed whole-exome sequencing and RNA sequencing of the two cell populations,respectively.Results:The immunophenotypes of pDCs in both patients were positive for CD123bri,HLA-DR,CD4,CD303,CD304,and negative for CD56,CD34,CD117,and TdT.The variant allele frequency of gene mutations in myeloid blasts and pDCs were similar.The expression data showed myeloid blasts clustered tightly with hematopoietic stem cells,and pDCs from patients clustered tightly with granulocyte-monocyte progenitors/common myeloid progenitor,rather than with pDCs from the GEO platform.Conclusion:Our study suggested that pDCs derived from the leukemic clone,evidenced by a shared mutation profile and similar transcriptional signatures between pDCs and concurrent myeloid blasts.

关键词： Acute myeloid leukemia Gene expression Mutation Plasmacytoid dendritic cells

Current treatment paradigm and survival outcomes among patients with newly diagnosed multiple myeloma in China:a retrospective multicenter study

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Cancer Biology & Medicine 2023年第1期20卷 77-87页

作者： Huishou Fan Weida Wang Ya Zhang Jianxiang Wang Tao Cheng Lugui Qiu Xin Wang Zhongjun Xia Gang An State Key Laboratory of Experimental Hematology National Clinical Research Center for Blood DiseasesHaihe Laboratory of Cell EcosystemInstitute of Hematology&Blood Diseases HospitalChinese Academy of Medical Sciences&Peking Union Medical CollegeTianjin 300020China state key laboratory of Oncology in South China Collaborative Innovation Center for Cancer MedicineSun Yat-Sen University Cancer CenterGuangzhou 510060China Department of hematology Shandong Provincial Hospital Affiliated to Shandong First Medical UniversityJinan 250021China

Objective:Evidence on the prognostic value of autologous stem cell transplantation(ASCT)and minimal residual disease(MRD)dynamics of patients with newly diagnosed multiple myeloma(NDMM)in China is limited.Our objective in the current study was to understand the current care paradigm and outcomes of these patients.Methods:This longitudinal cohort study used historical data from three top-tier hematologic disease care hospitals that contributed to the National Longitudinal Cohort of Hematological Diseases-Multiple Myeloma.Treatment regimens[proteasome inhibitor(PI)-,immunomodulatory drug(IMiD)-,PI+IMiD-based,and conventional],post-induction response,ASCT and MRD status,and survival outcomes[progression-free survival(PFS)and overall survival(OS)]were evaluated.Results:In total,454 patients with NDMM were included(median age,57 years;59.0%males)with a median follow-up of 58.7 months.The overall response rate was 91.0%,83.9%,90.6%,and 60.9%for PI-,IMiD-,PI+IMiD-based,and conventional regimens,respectively.Patients with ASCT during first-line therapy(26.2%)had a longer PFS and OS than patients who did not receive ASCT[median PFS,42.9 vs.21.2 months,P<0.001;median OS,not reached(NR)vs.65.8 months,P<0.001].The median OS was NR,71.5,and 56.6 months among patients with sustained MRD negativity,loss of MRD negativity,and persistent MRD,respectively(P<0.001).Multivariate analysis revealed that the lactic dehydrogenase level,International Staging System stage,extra-medullary disease,and upfront ASCT were independent factors in predicting OS among NDMM patients.Conclusions:Our study showed that novel agent-based regimens,first-line ASCT,and sustained MRD negativity were associated with a superior outcome for patients with NDMM in China(Identifier:NCT04645199).

关键词： Multiple myeloma autologous stem cell transplantation minimal residual disease survival outcomes multicenter study

Risk factors for CMV infection within 100 days posttransplantation in patients with acute leukemia

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Blood Science 2022年第3期4卷 164-169页

作者： Juan Chen Aiming Pang Yuanqi Zhao Li Liu Runzhi Ma Jialin Wei Xin Chen Yi He Donglin Yang Rongli Zhang Weihua Zhai Qiaoling Ma Erlie Jiang Mingzhe Han Jiaxi Zhou Sizhou Feng State Key Laboratory of Experimental Hematology National Clinical Research Center for Blood DiseasesHaihe Laboratory of Cell EcosystemInstitute of Hematology and Blood Diseases HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeTianjin 300020China

Objective:To investigate the risk factors for cytomegalovirus(CMV)infection within 100 days and the relationship between early CMV infection and 1-year relapse for patients with acute leukemia following allogeneic hematopoietic stem cell transplantation(allo-HSCT).Methods:Three hundred fifty-nine patients with acute leukemia who received allo-HSCT at our center between January 2015 and January 2020 were retrospectively reviewed.Results:Of 359 patients,48.19%(173)patients experienced CMV infection within 100 days posttransplantation.In univariate and multivariate logistic analysis,haploidentical-related donor(HRD)(P<0.001;odds ratio[OR],5.542;95%confidence interval[CI],3.186–9.639),and ratio of CD3^(+)CD8^(+)cells in lymphocytes<14.825%(P<0.001;OR,3.005;95%CI,1.712–5.275)were identified as 2 independent risk factors.One-year relapse rate(RR)between the CMV infection group and the non-CMV infection group was not statistically significant(18.5%vs 19.9%,P=0.688).When we divided the total cohort into AML,ALL,and MAL subgroups,there were no significant differences as well(P=0.138;P=0.588;P=0.117;respectively).Conclusion:In conclusion,donor type(HRD)and the insufficient recovery of CD3^(+)CD8^(+)cells were independent risk factors for CMV infection within 100 days posttransplantation in patients with acute leukemia.CMV infection within 100 days did not influence the incidence of relapse in 1 year for patients with acute leukemia.

关键词： Acute leukemia Allogeneic hematopoietic stem cell transplantation Cytomegalovirus Risk factors Relapse

Cytogenetic aberrations of lymphoplasmacytic lymphoma/ Waldenström’s macroglobulinemia in Chinese patients

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Chinese Medical Journal 2023年第10期136卷 1240-1242页

作者： Wenjie Xiong Tingyu Wang Ying Yu Yang Jiao Jiawen Chen Yi Wang Chengwen Li Rui Lyu Qi Wang Wei Liu Weiwei Sui Gang An Dehui Zou Lugui Qiu Shuhua Yi State Key Laboratory of Experimental Hematology National Clinical Research Center for Blood DiseasesHaihe Laboratory of Cell EcosystemInstitute of Hematology&Blood Diseases HospitalChinese Academy of Medical Sciences&Peking Union Medical CollegeTianjin 300020China Tianjin Institutes of Health Science Tianjin 301600China

To the Editor:As a rare indolent B cell non-Hodgkin lymphoma,lymphoplasmacytic lymphoma/Waldenström’s macroglobulinemia(LPL/WM)has unique clinical and biological characteristics.[1]However,due to the difficulties in obtaining tumor metaphase for karyotyping and slow cell proliferation,only very few studies have detected the cytogenetic aberration of LPL/WM.[2,3]In addition,6q deletion is the most common cytogenetic aberration in WM,with an incidence rate of about 50%.[2]Nevertheless,other cytogenetic aberrations remain largely unclear,and the prognostic role of cytogenetic aberrations needs to be further explored.In the present study,we systematically analyzed 305 LPL/WM cases in China,focusing on the characteristics and cytogenetic aberrations in Chinese patients.

关键词： lymphoma patients globulin