Multiple myeloma (MM) is an incurable malignancy. The treatment mainly includes induction therapy, minimal residual disease (MRD) clearance therapy, and maintenance therapy. For high-risk/ultra-high-risk (UHR) or relapsed/refractory MM, optimizing the eradication of MRD and sustaining long-term suppression of MRD at low levels are a formidable challenge. Ixazomib (I) is a reversible proteasome inhibitor (PI) that is available orally as the prodrug ixazomib citrate. Lisaftoclax is a novel, potent, selective BCL-2 inhibitor under clinical development for the treatment of patients with hematologic malignancies or solid tumors and has shown clinical antitumor benefit. Herein, we report two patients with relapsed/refractory UHR MM who achieved durable disease control with MRD negativity after receiving ixazomib, lisaftoclax, and dexamethasone (ILD) as maintenance therapy following B Cell Maturity Antigen BCMA-chimeric antigen receptor (CAR)-T cell therapy. Regarding the treatment regimen, all drugs were administered orally: ixazomib 4 mg d1, d8, and d15; lisaftoclax 400 mg d1-d14; and dexamethasone 20 mg d1, d8, and d15. One treatment cycle is defined as 28 days. Treatment will be discontinued in the event of uncontrollable active infection or organ injury. These cases demonstrate that the ILD combination therapy can optimize MRD eradication and sustain long-term MRD suppression, offering a promising therapeutic option for patients with limited treatment choices. Formal evaluations of this regimen in patients with high-risk/ultra-high-risk or relapsed/refractory MM may be meaningful. This study was supported by the China Cancer Foundation (Project No.: CFC2023WJZD003).

Relapse after allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains the leading cause of treatment failure in acute myeloid leukemia (AML), yet the non-coding RNA-mediated regulatory mechanisms in relapse-driving tumor clones are not fully understood. In this study, we performed transcriptomic profiling of CD34+ cells from the bone marrow of patients who had relapsed after allo-HSCT and from those in sustained remission, and observed that long non-coding RNAs (lncRNAs) exhibited the largest magnitude of differential expression relative to other RNA classes. To systematically elucidate their function, we integrated correlation analysis, neighborhood topology, and thermodynamic modeling to construct relapse-associated competing endogenous RNA (ceRNA) networks. These networks highlighted multiple lncRNA-miRNA-mRNA axes potentially promoting leukemic progression. Functional interrogation using CRISPR-based xenograft assays and a single-cell CRISPR screening platform (Perturb-seq-like strategy) revealed that relapse-associated lncRNAs regulate AML cell proliferation and survival. Among them, SNHG8 emerged as a representative regulator that promotes relapse through the SNHG8-miR-625-ZC3H13/15 signaling axis. SNHG8 knockdown markedly impaired AML cell growth and triggered apoptosis. Furthermore, treatment with hypomethylating agents (HMAs) such as azacitidine and decitabine differentially modulated lncRNA/ceRNA expression signatures, thereby linking clinical interventions to transcriptomic regulation. Together, our findings identify lncRNA-ceRNA networks in post-transplant relapse of AML and identify the SNHG8 axis as a potential therapeutic vulnerability, providing a rationale for further investigation of lncRNA-targeted strategies in this clinical setting.

Background: Acute myeloid leukemia (AML) is an aggressive and molecularly diverse hematologic malignancy with unfavorable clinical outcomes and limited options for targeted therapy. This study investigated whether polypyrimidine tract-binding protein 1 (PTBP1), an RNA-binding protein (RBP), affects AML progression by binding to WNK lysine-deficient protein kinase 1 (WNK1). Methods: We first determined the level of WNK1 in AML using the Gene Expression Profiling Interactive Analysis‌ (GEPIA) database and verified it by quantitative reverse transcription polymerase chain reaction (qRT-PCR) and Western blotting (WB) assay. AML cell migration and invasion were analyzed using Transwell assays following WNK1 modulation. Epithelial-to-mesenchymal transition (EMT) marker level was confirmed by WB assay. The influence of WNK1 on the in vivo metastasis of AML was verified via tail vein injection of WNK1-knockdown AML cells into Non-Obese Diabetic/Severe Combined Immunodeficiency (NOD/SCID) mice. Mechanistically, RNA pull-down and RNA immunoprecipitation (RIP) assays were utilized to interpret the relationship between PTBP1 and WNK1 and to determine whether PTBP1 affects AML cell migration and invasion by regulating WNK1, using rescue experiments. Results: WNK1 was highly expressed in AML. WNK1 inhibition hindered AML cell migration, invasion, and the expression of EMT markers. WNK1 depletion markedly suppressed the metastasis of AML cells in vivo. Mechanistically, PTBP1 directly bound to WNK1 and increased its mRNA stability. Furthermore, PTBP1 facilitated AML cells migration, invasion, and the expression of EMT markers via WNK1. Conclusion: We demonstrate that PTBP1 promotes AML progression by modulating WNK1. PTBP1 may therefore represent a potential therapeutic target in AML.

Outcomes in older patients with Hodgkin lymphoma (HL) are compromised by the interplay of patient frailty and disease aggressiveness; however, current stratification tools, including the International Prognostic Score (IPS) and Comprehensive Geriatric Assessment (CGA), lack sufficient prognostic discrimination in the older HL population. We developed and validated a prognostic model integrating metabolic tumor burden and geriatric assessment. In this retrospective study across 14 centers in China between 2006 and 2024, we enrolled 306 patients aged ≥ 60 years with histologically confirmed HL. Patients were randomly partitioned into training (n = 250) and validation (n = 56) cohorts. Among the 306 patients, 98 deaths and 130 progression events were recorded during follow-up. We analyzed 21 candidate variables. The least absolute shrinkage and selection operator (LASSO) analysis and multivariable Cox regression identified five independent predictors for 5-year overall survival (OS): age > 73 years, baseline 18F-FDG PET/CT-derived total lesion glycolysis (TLG) > 200, hemoglobin ≤ 101 g/L, activities of daily living (ADL) dependence, and lymphocyte-depleted classical Hodgkin lymphoma (LDCHL). A weighted Geriatric Risk Score stratified patients into low-, intermediate-, and high-risk groups. In the validation cohort, the Geriatric Risk Score showed higher discriminatory accuracy than the CGA for both OS (C-index, 0.770 [95% CI, 0.674-0.867] vs. 0.671 [0.581-0.762]) and PFS (0.761 [0.668-0.853] vs. 0.635 [0.535-0.735]). Among advanced-stage (Ann Arbor stage III-IV) patients, it also outperformed the IPS-7 and IPS-3. By integrating metabolic tumor burden and geriatric assessment, the Geriatric Risk Score improves risk stratification over existing tools in older patients with HL.

Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) is the most common histological subtype of PTCL. PTCL-NOS has a relatively low incidence, and its pathogenesis and mechanisms of drug resistance remain unclear, leading to lagging research progress. The aim of the present study was to summarize the clinical features and outcomes of patients with PTCL-NOS. A total of 30 patients with treatment-naive PTCL-NOS who were admitted to The Second Hospital of Hebei Medical University (Shijiazhuang, China) between September 2013 and September 2023 were retrospectively analysed. The median age at diagnosis was 59 years (range, 17-70 years), and the male-to-female ratio was 2.75:1. The median follow-up duration was 59 months. The 3-year overall survival (OS) and progression-free survival (PFS) rates were 50.4 and 28.9%, respectively. Multivariate analysis showed that an Eastern Cooperative Oncology Group performance status >1, bone marrow involvement and a platelet count <150×109/l were independent risk factors for OS, whereas bone marrow involvement and albumin levels <35 g/l were independent risk factors for PFS. There was no significant difference between the cyclophosphamide, doxorubicin, vincristine, prednisone and etoposide regimen and the cyclophosphamide, vincristine, doxorubicin and prednisone regimen, whereas combinations with chidamide showed a trend toward an improved PFS. Within the cohort, 2 patients with relapsed and refractory CD30-positive PTCL-NOS received salvage chemotherapy with brentuximab vedotin (BV), a monoclonal antibody, and achieved complete metabolic remission, followed by sequential allogeneic haematopoietic stem-cell transplantation, resulting in long-term sustained remission. In conclusion, patients diagnosed with PTCL-NOS generally have a poor prognosis. Nevertheless, the use of innovative targeted therapies, such as chidamide and BV, shows potential to improve treatment outcomes in these patients.

Acute myeloid leukemia (AML) with chromosomal translocation t(8;21)(q22;q22.1) is a rare subtype, accounting for 4-8% of all cases of AML. Despite its rarity, it has a favorable outcome. The translocation event culminates in the formation of the Runt-related transcription factor 1 (RUNX1)::RUNX1 partner transcriptional co-repressor 1 (RUNX1T1) fusion protein, which is implicated in hematopoietic differentiation and maturation. Furthermore, monoclonal gammopathy of undetermined significance (MGUS) is characterized by the presence of monoclonal immunoglobulins in the blood or urine, serum M protein level of <3 g/dl and <10% clonal plasma cells in the bone marrow, with no accompanying end-organ damage associated with myeloma. The simultaneous occurrence of AML and MGUS is exceedingly rare. The present report describes the case of a male patient with AML and a RUNX1::RUNX1T1 fusion gene, not arising from the usual chromosomal translocation but rather from a complex translocation event involving t(8;17;21) (q22;q24;q22). The patient achieved complete remission (CR) following an idarubicin (12 mg/m2, days 1-3) + cytarabine (100 mg/m2, d1-7) regimen chemotherapy. Subsequent bone marrow monitoring revealed CR of AML during consolidation chemotherapy; however, ~5% of plasma cells were detected in the bone marrow. Flow cytology confirmed the presence of monoclonal plasma cells, and a positive hematuria immune-fixed electrophoresis assessment led to a diagnosis of MGUS. Due to economic constraints, the patient and their family declined high-dose cytarabine-based combination chemotherapy and hematopoietic stem cell transplantation, opting instead for intermittent use of standard doses of anthracycline combined with cytarabine maintenance therapy. The disease relapsed after 10 months, the patient discontinued treatment and died shortly after.

Background Elderly patients (≥60 years) with classical Hodgkin lymphoma (cHL) experience disproportionately poor outcomes, with historical 5-year overall survival (OS) rates of 50% globally due to treatment-related toxicity, comorbidities, and disease biology. In China, this population faces additional challenges including delayed diagnoses and limited access to novel therapies-brentuximab vedotin (BV) and PD-1 inhibitors were only approved in 2020 and 2019, respectively. Real-world data on treatment evolution and survival impacts in elderly Chinese cHL patients, particularly those with high comorbidity burdens (CIRS-G ≥10 prevalence >30% in prior studies), remain critically scarce. This 15-year multicenter analysis aims to define the survival benefit of novel agents and identify key prognostic factors in this vulnerable cohort. Methods We conducted a retrospective study of 493 consecutive newly diagnosed cHL patients ≥60 years treated at 13 tertiary centers across China (2008-2023). Inclusion required pathological confirmation and ≥6-month follow-up. Collected variables: 1) Baseline characteristics: age, Ann Arbor stage, B-symptoms, ECOG PS; 2) Geriatric metrics: CIRS-G comorbidity index (severe: ≥10), Activities of Daily Living (ADL) scale (impairment: score <6); 3) Treatment details: frontline/salvage regimens (chemotherapy/radiotherapy/novel agents), BV/PD-1 utilization timelines; 4) Response and toxicity: PET-CT parameters (Deauville, total metabolic tumor volume [TMTV] available in 336 patients [68%]), grade 3-5 adverse events (CTCAE v5.0); 5) Molecular profiling: 475-gene next-generation sequencing panel in 158 patients (32%). Survival endpoints (OS/PFS) were compared between novel-agent-containing (BV±PD-1±chemo) and chemo-only groups using Kaplan-Meier/log-rank tests. Multivariable Cox regression identified OS predictors adjusting for age, stage, CIRS-G, and ADL. Biomarker correlations used logistic regression. Results The cohort (median age 72 years, 58% male) exhibited high-risk features: 64% stage III/IV, 48% with ≥3 comorbidities (CIRS-G≥10: 31%), and 22% ADL impairment. Treatment patterns shifted dramatically post-2020: BV utilization increased from 0 (pre-2020) to 21% (2020-2023), while PD-1 inhibitor use rose from 1% to 27% (P<0.001). Frontline regimens included ABVD (43%), AVD (20%), BV-AVD (8%), and PD-1±chemo (7%). Landmark survival analysis demonstrated transformative benefits with novel agents: 3-year OS was 78% (95%CI 72-84) for novel-agent recipients (n=142) versus 53% (95%CI 47-59) for chemo-only patients (n=351) (HR=0.45, P<0.001); 3-year PFS was 65% (59-71) vs 38% (32-44) (HR=0.49, P<0.001). This OS advantage persisted in high-risk subgroups: age >75 years (HR=0.52, P=0.008) and CIRS-G≥10 (HR=0.57, P=0.01). Overall cohort median OS was 58 months (5-year OS 46%), with multivariable analysis confirming independent OS predictors: age >75 years (HR=2.1, P=0.001), ADL impairment (HR=2.8, P<0.001), and ≥3 comorbidities (HR=1.9, P=0.01). Grade ≥3 toxicity was lower with novel agents (38% vs 52% for chemo-only, P=0.03), though BV-related neuropathy was more frequent (18% vs 6%, P<0.001). Exploratory biomarker analysis revealed high TMTV (>80 cm³) predicted inferior PFS (HR=1.8, P=0.02), and TP53 mutations (21%) associated with primary refractoriness (OR=3.1, P=0.004). Conclusion This largest real-world study of elderly Chinese cHL establishes that BV and PD-1 inhibitors confer unprecedented survival improvements (25% absolute 3-year OS gain), fundamentally altering treatment paradigms for this high-risk population. The survival benefit extends to traditionally excluded subgroups (>75 years, high comorbidity burden), supporting frontline integration of novel agents in fit patients. Geriatric assessments (ADL/CIRS-G) outperform conventional staging in predicting mortality risk, underscoring their essential role in treatment stratification. While toxicity profiles differ from chemotherapy, novel regimens demonstrate overall better tolerability. Emerging biomarkers (TMTV, TP53) offer pathways for personalized therapy-a critical need given the 46% 5-year OS rate with conventional approaches. Prospective trials optimizing novel-agent combinations guided by geriatric and molecular metrics are urgently warranted.

Acute Myeloid Leukemia (AML) carries a high mortality rate in elderly patients who often face limited treatment options and a poor overall prognosis. The combination of Venetoclax (VEN) and Decitabine (DEC) is recommended for treatment, yet there is insufficient evidence to fully support its efficacy. This study aims to perform a meta-analysis to evaluate the effectiveness and safety of the VEN + DEC regimen in treating elderly patients with AML. We systematically searched PubMed, EMBASE, the Cochrane Library, CNKI, and WanFang. Efficacy was evaluated using complete remission (CR), composite response rate, overall response rate, and median overall survival. Safety was assessed based on adverse events. The fixed/random effect model was employed to evaluate the effect sizes. Seven articles were included in this meta-analysis. VEN + DEC group (OR 1.90, 95

Hematological malignancies are a diverse group of cancers that originate in the blood and bone marrow and are characterized by the abnormal proliferation and differentiation of hematopoietic cells. Myeloid blasts, which are derived from normal myeloid progenitors, play a central role in these diseases by disrupting hematopoiesis and driving disease progression. In addition, other myeloid cells, including tumor-associated macrophages and myeloid-derived suppressor cells, adapt dynamically to the tumor microenvironment, where they can promote immune evasion and resistance to treatment. This review explores the unique characteristics and pathogenic mechanisms of myeloid blasts, the immunosuppressive roles of myeloid cells, and their complex interactions within the TME. Furthermore, we highlight emerging therapeutic approaches targeting myeloid cells, focusing on strategies to reprogram their functions, inhibit their suppressive effects, or eliminate pathological populations altogether, as well as the latest preclinical and clinical trials advancing these approaches. By integrating insights from these studies, we aim to provide a comprehensive understanding of the roles of myeloid cells in hematological malignancies and their potential as therapeutic targets.

OBJECTIVE:To understand the etiology, clinical characteristics and prognosis of secondary hemophagocytic syndrome (HLH), so as to improve the understanding of HLH and reduce the rates of misdiagnosis and missed diagnosis of HLH. METHODS:A retrospective study was conducted to analyze the cause, clinical characteristics, laboratory findings, therapy and outcomes of 75 adult patients with secondary HLH admitted to our hospital from January 2015 to December 2021. Follow-up continued until the last discharge time. RESULTS:Among 75 patients, infection-related HLH was the most common (45.33%), followed by lymphoma-related HLH (17.33%). Fever was the most common clinical manifestation (97.67%). Laboratory indicators such as NK cell activity (98.31% low or absent), sCD25 (93.22% increased), and serum ferritin (94.44% elevated) had higher sensitivity in diagnosis. By comparing the clinical manifestations and laboratory indicators of HLH patients with different causes, sex, lymph node enlargement and bone marrow morphology were more valuable for the diagnosis of primary disease (all P <0.05). By comparing the treatment and clinical outcomes of HLH patients with different causes, the highest clinical remission rate (83.3%) was achieved in patients with autoimmune disease-related HLH treated with hormone+cyclosporine (P <0.05). The overall 12-month survival rate of all patients was 26.7%, in which the infection-related HLH was the lowest (14.7%) while autoimmune disease-related HLH was the highest (63.6%). CONCLUSION:The causes and clinical characteristics of adult secondary HLH are varied, with poor prognosis and heterogeneity in disease severity. It is important to identify HLH cause early for diagnosis and needed to further understand HLH.

Abstract Selinexor is an oral, reversible, potent Selective Inhibitor of Nuclear Export / SINE TM compound that specifically blocks the protein Exportin 1 (XPO1), also known as chromosomal region maintenance 1 (CRM1). Selinexor induces cell cycle arrest, inhibits DNA damage repair and modulates the expression of NF-κB in cancer cells lines and has demonstrated broad activity across several hematologic malignancies including AML. In preclinical studies, AML cells, when treated with selinexor, exhibited sensitivity to chemotherapy, in part, by blocking the nuclear export of topoisomerase II (Clin Cancer Res 2016;22(24):6142-6152). This, when coupled with the presence of an anthracycline antibiotic, resulted in an increased number of DNA strand breaks. In May 2019, the FDA approved the use of selinexor in combination with dexamethasone for adults with relapsed or refractory multiple myeloma who received at least four prior therapies with disease refractory to at least two immunomodulatory agents, at least two proteasome inhibitors, and a CD38-targeting monoclonal antibody. The aim of this study is to evaluate the efficacy and safety of the combination therapy of Decitabine, Venetoclax， and Selinexor (DVS) in the treatment of newly diagnosed AML and MDS patients who are not suitable for intensified chemotherapy, as well as in patients with relapsed/refractory AML and high-risk MDS. Method: This multicenter, single arm, prospective clinical study conducted at multiple research centers in China. A total of 96 patients will be enrolled in the study. The patients were treated with Decitabine 20 mg/m2, d1-5；Venetoclax 100 mg d1, 200 mg d2, 400 mg d3-21; Selinexor 60 mg/d, d4, 11, 18. Every 28 days are one cycle. The primary endpoints are complete remission(CR)/complete remission with incomplete count recovery (CRi) / incomplete recovery of peripheral blood cell count/ANC>0.5X109/L (500/uL) and platelet count ≥ 50 × 109/L (50000/uL), meeting other CR criteria(CRh)/ morphologic leukemia-free state (MLFS) (CR/CRI/CRh/MLFS). Secondary endpoints include objective response rate (ORR) (complete response rate/incomplete recovery of peripheral blood cell count, partial response rate) ，safety. Result: Until July, 2024, there are 14 patients(AML/MDS：12/2) included this study. The median age of the patients is 63.5（24-76）years. The male and female are 5 and 9. Among them, The median of white blood cell count (WBC) is 3.3×10^9/L, platelet count (Platelets) is 41.5×109/L, and the percentage of blast cells in bone marrow is 29.25%. During the first course of treatment, 2 patients discontinued the treatment due to adverse events. 9 patients were evaluated for efficacy, the other three cases did not enter the first course of evaluation due to short follow-up time. With a median follow-up of survivors of 40(27-117) days, 9 patients' efficacy can be evaluated. The complete remission rate was 55.56%（5/9），One （11.11%）patient has stable disease. Five patients experienced adverse events, including two cases of pulmonary infection, one case of thrombocytopenia, one case of pancytopenia, and one case of diarrhea. All were mild or moderate in severity. Conclusion：Combination therapy of Decitabine, Venetoclax，and Selinexor (DVS) has a manageable safety profile and showed activity in AML and MDS.

The occurrence of acute myeloid leukemia (AML) with a simultaneous diagnosis of breast cancer (BC) is rarely reported in the literature. The present study reports the case of a 50-year-old female patient diagnosed with AML coexisting with metastatic BC. Following one cycle of treatment with azacytidine in combination with oral venetoclax for AML, the patient achieved complete remission with incomplete hematological recovery. In addition, the mass in the left breast was smaller following adjuvant chemotherapy. However, due to a refusal from the patient to accept an allogeneic hematopoietic stem cell transplantation (allo-HSCT), the patient succumbed 3 months after diagnosis due to septic shock from neutropenia following the third cycle of chemotherapy. Altogether, the present case report highlighted the application of venetoclax, an oral selective B-cell lymphoma-2 inhibitor, both in hematologic malignancies and solid neoplasms, as an effective therapeutic regimen. Considering the fatality rate associated with AML, allo-HSCT is the only available strategy that can be used to achieve the long-term survival of patients with AML and BC.

This study aimed to determine the expression levels of the autophagy markers Beclin-1 and p62 in patients with diffuse large B-cell lymphoma (DLBCL) and explore the association between autophagy and disease prognosis. The expression of Beclin-1 and p62 was investigated in patients with DLBCL (n=60) and patients with reactive lymphoproliferative disease (RLD; n=20) using immunohistochemistry. The association between the clinical characteristics of patients with DLBCL and autophagy status was further analyzed. Beclin-1 levels were increased in patients with RLD compared to those with DLBCL, but the difference was not statistically significant (P>0.05). p62 levels in patients with DLBCL were significantly higher than those in patients with RLD (P<0.05). Beclin-1 expression was associated only with the Ann Arbor stage (P<0.05), whereas p62 expression was associated with the Ann Arbor stage, International Prognostic Index score, extranodal involvement, and Ki-67 index (P<0.05). Beclin-1 and p62 levels were not associated with short-term treatment efficacy in patients with DLBCL. Survival analysis showed that Beclin-1 expression had no significant effect on 2-year progression-free survival (PFS) or overall survival (OS) (P>0.05). However, high p62 expression in patients with DLBCL was associated with reduced 2-year PFS compared with that of patients with low p62 expression (P<0.05); the 2-year OS was not affected (P>0.05). Our results demonstrate that autophagic activity affects the prognosis of patients with DLBCL; the lower the autophagic activity, the shorter the PFS. Targeted p62 knockout may be a novel therapeutic strategy for the treatment of patients with DLBCL.

Background: PTCL-NOS is the most common histological subtype of PTCL, characterized by high heterogeneity in clinical presentation, immunophenotype, and genetic features. The incidence of PTCL-NOS is regional distributed, with PTCL accounting for 23-27% of non-Hodgkin lymphomas in China, significantly higher than the 10% in Western countries. In terms of treatment, CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) like regimens are still the first-line therapy; However, due to the high invasiveness and recurrence rate of the disease, the five-year progression free survival (PFS) rate is only 29%. In recent years, with the application of new drugs such as histone deacetylase (HDAC) inhibitor Chidamide and antibody conjugated drugs brentuximab-vedotin (BV) etc, certain clinical benefits have been brought to PTCL patients, however these patients still faces significant survival challenges. Aim:The aim of this study was to explore the clinical features of Chinese patients with PTCL-NOS in a single institution, to identify potential prognostic factors, and to provide references for early diagnosis and improvement of long-term survival Methods: Thirty patients with treatment-naïve PTCL-NOS admitted to the Second Hospital of Hebei Medical University from January 2015 to 2023 were analyzed retrospectively. Results: A total of 30 patients were included.The median age of the patients at diagnosis was 59 years old (range: 17~70) , and the male to female ratio was 2.75:1. Most patients were an advanced stage at diagnosis, with intranodal primary as the main type; in terms of immunophenotype, there was a variable loss of pan-T cell markers, most commonly seen in abnormal expression of CD5 and CD7. The CD30 positivity rate in this study was 53.8%, which is higher than previous literature reports, or may be related to the different cutoff values of CD30 positive and a relatively small sample size of this study. This suggests that these patients with CD30 positive can use BV as a preferential therapy choice.The follow-up period ended in September 2023, with a median follow-up time of 59.0 months. The 3-year OS and PFS were 50.4% and 28.9%, respectively. The overall 5-year overall survival (OS) and 5-year PFS were 44.8% and 21.7%, respectively. Multivariate analysis showed that ECOG score >1, bone marrow involvement, and PLT <150×109/L were independent risk factors for OS, while bone marrow involvement and ALB <35g/L were independent risk factors for PFS. In terms of treatment, there was no significant difference between the CHOPE regimen and the CHOP regimen, and the combination with Chidamide showed a trend towards improved OS and PFS. Two relapsed and refractory patients wtith CD30 positive had received salvage chemotherapy based on BV monoclonal antibody and achieved complete metabolic remission, followed by sequential allogeneic hematopoietic stem cell transplantation and obtained long-term sustained remission. Conclusion: Patients with PTCL-NOS in China have a poor prognosis, and the conventional CHOP-like regimens has limited efficacy. Several novel drugs such as Chidamide and BV may improve patient prognosis in some individual patients. Future research should focus on the development of effective clinical prediction tools and treatment strategies, particularly accelerating the development of novel drugs for PTCL

8p11 myeloproliferative syndrome (EMS) is a rare and aggressive hematological malignancy, characterized by myeloproliferative neoplasms, and associated with eosinophilia and T- or B-cell lineage lymphoblastic lymphoma. The pathogenesis is defined by the presence of chromosomal translocations associated with the fibroblast growth factor-1 (FGFR1) gene, located in the 8p11-12.1 chromosomal locus. At present, only ~100 cases have been reported globally. At least 15 partner genes have been identified, including the most common, the zinc finger MYM-type containing 2 (ZNF198)-FGFR1 fusion gene formed by t(8;13)(p11;q12). Different fusion genes determine the clinical manifestations and prognosis of the disease. Patients with EMS with t(8;13)(p11;q12) commonly present with lymphadenopathy and T-lymphoblastic lymphoma, which usually converts to acute myeloid leukemia (AML) with the progression of the disease. The present study describes the case of an elderly female patient with EMS with t(8;13)(p11;q12), presenting with myeloid/lymphoid syndrome (myeloproliferative neoplasms and T lymphoblastic lymphoma). The patient received the CHOPE regimen combined with tyrosine kinase inhibitor (dasatin) treatment and obtained short-term complete remission. However, 6 months later, the disease progressed from EMS to AML and the patient died due to ineffective induction therapy. The present study also reviews the relevant literature about this unusual entity to enhance the understanding of EMS.

Background: Ocular adnexal marginal zone B-cell lymphoma (OAML) is a rare entity of indolent lymphoma with lesions confined to the ocular adnexal, although few patients also have extraocular involvement. Recently, the Chinese Ocular Lymphoma Collaborative Group (COLCG) published “Chinese expert consensus on the diagnosis and management of ocular adnexal extranodal marginal zone mucosa-associated lymphoid tissue lymphoma”. To better provide guidance for improved treatment of OAML, we conducted a multicenter study including 694 patients to demonstrate the real-world data concerning clinical characteristics, treatments, and long-term outcomes. Methods A total of 694 patients with OAML from 11 centers of China were included. Baseline characteristics, treatments, long-term outcomes and treatment-related adverse events were collected. This study was approved by the Ethics Committee of Beijing Tongren Hospital. Results The median age was 57 years old (13-91). 33 patient had an ECOG-PS score of ≥2, and 25 patients had systemic B symptoms. 12 patients reported baseline auto-immune diseases, mainly sicca syndrome and systemic lupus erythematosus. 42 patients had previous diagnosis of eye diseases, mainly cataract and xerophthalmia. 17 patients had a past medical history of non-hematologic malignancies and had stable diseases at the time of OAML diagnosis. 29 patients carried infectious diseases, mainly hepatitis B or C. Because chlamydia psittaci was not routinely tested in China, only one patient was found to be positive for chlamydia psittaci. The diagnosis of OAML was confirmed by surgical resection or biopsy of ocular lesions. PET-CT and/or MRI scan were performed to stage the disease. 144 patients had bilateral ocular disease (20.7%), and the frequency of affected ocular lesions from most to least were bulbar conjunctiva, eyelid, lacrimal gland, optic nerve, and paranasal sinus, etc. Only one patient had intracranial involvement by direct lymphoma infiltration. Bone marrow biopsy was performed in 217 patients, among whom 16 patients were defined positive for bone marrow infiltration of lymphoma (7.4%), and the baseline PET-CT scan could not predict the bone marrow status for these patients. Using Ann-Arbor staging system (data was available for 524 patients), 421 patients had stage I disease (80.3%), 30 patients had stage II disease (7.1%), and 73 patients had advanced diseases. Due to the heterogeneity of ocular lesions, TNM staging system was also used for OAML patients. 156 patients were staged as T1, 295 patients were staged as T2, and the remaining patients were staged as T3 or T4. After diagnosis of OAML, 229 patients underwent a regular watch and wait (W&W) strategy due to no residual lymphoma or no symptoms. 160 patients (34.4%) received radiotherapy (RT). 187 patients (40.2%) were treated with immunochemotherapy regimens, such as R-CHOP, R-CVP, BR, etc. Local ocular injection of rituximab was done in eight patients. Best response evaluation was available for 455 patients who received post-surgery consolidation therapy. The overall response rate (ORR) was 94.5% (including 237 patients with complete response and 193 patients with partial response), and five patients had progressive disease. At a median follow-up time of 731 days (12-7443), a total of 142 patients got disease progression or relapse (20.5%), among whom 130 patients had intraocular relapse and 12 patients had extraocular relapse (8.5%). Only one patient was defined as histological transformation to DLBCL. 47 patients were treated with salvage RT, among whom 45 patients responded (95.7%, including 23 patients with complete response). 18 patients died, among whom only three patients died from lymphoma-related events. The 10-year overall survival rate was 95.5%, and 10-year lymphoma-specific overall survival rate was 99.3%. Concerning adverse events for patients who received RT, 33 developed dry eye syndrome (20.6%), 16 patients had diminution of vision (10%), and only two patients got cataract (1.3%). Conclusions: Chinese patients with OAML had favorable long-term outcomes, and the risk of histological transformation was very low. The application of RT as front-line consolidation strategy is insufficient, and the side effects of RT were tolerable. Future studies should focus on use of low-dose RT as upfront therapy for OAML to further reduce the relapse rate and improve the quality-of-life.

Objective To investigate the changes and clinical significance of the expression of neutrophil gelatinase-associated li-pocalin(NGAL)and autophagy during different time of renal ischemia/reperfusion(I/R)injury in rats.Methods The rat renal I/R in-jury model was established,thirty male Wistar rats were divided by random number table method into sham operation group(Sham group)and I/R group,according to the different time of reperfusion after I/R injury,the I/R group was divided into 4subgroups:2h group,6h group,24h group,48h group.Blood,urine and renal tissue samples were collected at different time points,NGAL levels of blood and u-rineweredetectedbyenzyme-linkedimmunoadsordentassay(ELISA)method.Bloodureanitrogen(BU)and serum creatinine(SCr)were detected by automatic biochemical analyzer.Hematoxylin-eosin staining was used to detect the degree of histopathological injury and score the degree of injury.TdT-mediated dUTP nick end labeling(TUNEL)method was used to detect the apoptosis of renal tubular ep-ithelial cells;the expression of autophagy related genes LC3 and Beclin-1gene was detected by real-time quantitative polymerase chain reaction(RT-qPCR)method.Results The expression levels of NGAL in blood and urine were elevated in the 2h after I/R injury,and peaked at 6h of reperfusion,and showed a downward trend at 24h.BU and Scr values began to increase after the 6h reperfusion of I/R in-jury and peaked at 24h of reperfusion.TUNEL positive cells began to increase at 6h after I/R injury,the number of the highest was at 24h reperfusion of I/R injury.Hematoxylin-eosin staining showed that there were different degrees of swelling and necrosis of renal tubular epithelial tissue in all groups after I/R injury.The expression of LC3 and Beclin-1 gene began to increased after the 6h reperfusion of I/R injury and peaked at 24h of reperfusion.Conclusion The expression level of NGAL increased at the early stage of renal I/R injury in rats,which was earlier than the changes of BU and SCr levels,and could be used as a molecular indicator for early diagnosis of renal I/R injury.During the aggravation of renal I/R injury,the expression of NGAL was increased and autophagy was activated,which indica-ted that NGAL and autophagy played a certain role in the process of renal I/R injury.

Plasma cell leukemia (PCL) is a plasma cell proliferative disorder with strong invasiveness, rapid progression and poor prognosis. The incidence of PCL is about (0.04-0.05)/100 000 per year. According to the multiple myeloma (MM) history, PCL can be divided into primary plasma cell leukemia (PPCL) and secondary plasma cell leukemia (SPCL). PPCL accounts for about 60% of PCL, and it is in the stage of leukemia at diagnosis and has no history of MM. SPCL accounts for the remaining 40% of PCL, and mostly shows as the MM end-stage manifestation, but also can be secondary to Waldenstrom macroglobulinemia, B-cell lymphoma, chronic lymphoblastic leukemia, amyloidosis, etc. Patients who progress from MM to SPCL account for 2%-4% of all MM patients. Due to the low incidence and strong clinical heterogeneity of PCL, the evidence-based medicine about PCL is relatively lacking, this article reviews the clinical characteristics of PCL and progress in its diagnosis and treatment.

Objective: This study intended to investigate the therapeutic effect of decitabine and thalidomide on myelodysplastic syndrome (MDS), immunological effect and effective mesenchymal stem cells (MSCs). Methods: Altogether 62 patients with MDS diagnosed in our hospital were selected. Patients who received 5-day treatment mainly and received decitabine from the 1st day to the 5th day were collected as group A (A), while patients who received thalidomide 1st to 5th day as in group A were collected as group B (B). The immunologic effects, blood and bone marrow index levels, clinical effects and adverse reactions of group A and group B before and after intervention were observed. Results: Th17 in the two groups after intervention were evidently lower than that before intervention, and the decrease of Th17 cells in group B after intervention was more obvious than that in group A (P<0.001). Th22 cells in the two groups after intervention were evidently down-regulated compared with those before intervention, and the down-regulation of Th17 cells in group B after intervention was more obvious than that in group A (P<0.001). However, compared with group A, the levels of CD3+, CD4+, CD4+/CD8+ in serum of group B increased more obviously and CD8+ decreased more obviously after intervention. The white blood cell count of group B after intervention was evidently higher than that of group A (P<0.001). The hemoglobin concentration after intervention in group B was evidently higher than that in group A (P<0.001). The platelet count after intervention in group A was evidently higher than that in group B (P<0.001). The total effective rate in group B was evidently higher than that in group A (P<0.05). Conclusion: The combination of decitabine and thalidomide has a better regulatory role in the immunological mechanism and bone marrow mesenchymal stem cells of patients with MDS than the single decitabine therapy on the premise of ensuring clinical efficacy.

目的 探讨自噬相关蛋白Beclin-1和p62在弥漫大B细胞淋巴瘤(DLBCL)中的表达情况,分析两者表达水平与DLBCL患者预后的关系.方法 回顾性分析2015年1月至2018年12月60例DLBCL患者的临床资料,并选取20例反应性淋巴增殖性疾病(RLD)患者作为对照.采用免疫组化法检测Beclin-1和p62蛋白的表达水平,对比分析两者表达水平与预后的关系.结果 Beclin-1在DLBCL及RLD组织中的高表达率分别为63.3％(38/60)和90.0％(18/20),差异有统计学意义(P=0.026).p62在DLBCL及RLD组织中的高表达率分别为70.0％(42/60)和30.0％(6/20),差异有统计学意义(P=0.003).Beclin-1表达水平仅与Ann Arbor分期有关(P=0.000),与年龄、性别、B症状、结外浸润、IPI积分、Bcl-2表达、Ki-67指数和分子亚型等均无关(P>0.05).p62表达与Ann Arbor分期、IPI积分、结外浸润、Ki-67指数有关(P<0.05),与年龄、性别、Bcl-2表达和分子亚型均无关(P>0.05).Beclin-1高低表达组(89.4％vs.59.1％,P=0.009)之间有效率的差异有统计学意义,而p62高低表达组之间(83.3％vs.76.2％,P=0.736)的差异无统计学意义.全组2年无进展生存率为58.3％,2年生存率为91.7％.进一步分析显示,Beclin-1高表达组和低表达组2年无进展生存率(48.1％vs.34.5％,P=0.311)和2年生存率(97.5％vs.95.5％,P=0.914)的差异均无统计学意义.p62高表达组和低表达组2年无进展生存率的差异有统计学意义(39.8％vs.66.3％,P=0.036),而2年生存率的差异无统计学意义(91.7％vs.100.0％,P=0.163).结论 肿瘤细胞的自噬状态影响DLBCL患者的预后,自噬水平越低,其PFS越短.