Worldwide, the diagnosis and treatment of immune thrombocytopenia (ITP) and Henoch-Schönlein purpura (HSP) remain a major and ongoing challenge in hematology. Emerging clinical evidences suggest serum mineral elements are associated with ITP or HSP, but the causal relationship between them is still unclear. Conducting a two-sample, bidirectional Mendelian randomization (MR) study to evaluate the causal association between serum mineral elements including zinc, copper, magnesium, iron and calcium with ITP and HSP. In this two-sample, bidirectional MR study, summary statistics data of genome-wide association studies (GWAS) on exposures including zinc, copper, iron, magnesium and calcium were extracted from the MRC-Integrative Epidemiology Unit (MRC-IEU). The GWAS data on study outcomes, including ITP and HSP, were obtained from the FinnGen consortium. MR-Egger intercept and MR-PRESSO global test were utilized to assess the heterogeneity and horizontal pleiotropic of instrumental variables (IVs) between the exposures and outcomes, respectively. Inverse variance weighted (IVW) test was used as the primary analysis method to evaluate the causal between serum mineral elements with the risk of ITP and HSP, and weighted-median, weighted model, MR steiger, MR-PRESSO and radial MR were used as auxiliary analysis methods, moreover, the odds ratio (OR) and 95

Polycomb repressive complex 2 (PRC2) is a multi-subunit complex that catalyzes the tri-methylation of histone H3 at lysine 27 (H3K27me3), serving as an epigenetic marker of gene silencing. PRC2 plays a crucial role in numerous fundamental biological processes, and its dysregulation is closely linked to cancer and developmental disorders. EZH2, a key component of PRC2, is aberrantly overexpressed in various human cancers. Inhibition of EZH2 enzymatic activity has been shown to effectively reduce cancer cell proliferation and tumorigenesis. Consequently, EZH2 is widely recognized as a driver of cancer, and the development of EZH2-specific inhibitors has become an active area of research. In this study, we screened over 2000 compounds from solid libraries using a PRC2 enzymatic activity assay and identified pyrroloquinoline quinone (PQQ) as a potent inhibitor of PRC2 methyltransferase activity in vitro. We evaluated the antitumor effects of PQQ across different tumor cell lines and found that it exhibited strong anticancer activity, specifically against B-cell lymphoma cells, which demonstrate elevated EZH2 activity. We used a combination of biochemical assays, cellular assays, and molecular docking studies to thoroughly investigate the inhibitory effects of PQQ on PRC2 activity. Furthermore, PQQ is a naturally occurring compound with various biological activities, including antioxidant and neuroprotective effects, and it has been approved as a nutritional supplement and health product in the United States. This study demonstrates, for the first time, that PQQ, a dietary supplement, selectively inhibits PRC2 methyltransferase activity, therefore providing new insights for targeted anti-lymphoma therapies involving PRC2.

Acute monocytic leukaemia, a subtype of acute myeloid leukaemia (AML), is a highly aggressive malignancy characterised by a poor prognosis, primarily due to the ability of leukaemic cells to evade immune surveillance. In this study, we demonstrate that homoharringtonine (HHT), an FDA-approved therapeutic agent for chronic myeloid leukaemia (CML), inhibits this immune evasion by targeting the FTO/m6A/LILRB4 signalling pathway in monocytic AML. Utilising RNA sequencing (RNA-seq) and various functional assays, we reveal that HHT treatment significantly reduces LILRB4 expression at both the RNA and protein levels, suggesting that the effects of HHT on LILRB4 are distinct from its well-established role as a protein synthesis inhibitor. Mechanistically, HHT treatment markedly increases global levels of RNA m6A in THP-1 cells by promoting the degradation of FTO, which subsequently diminishes the expression of its downstream targets, MLL1 and LILRB4. Furthermore, in vitro and in vivo analyses employing monocytic AML cell lines, mouse-derived AML xenograft models, and patient samples collectively support the conclusion that HHT suppresses immune evasion in monocytic AML by reducing LILRB4 expression. Importantly, the downregulation of LILRB4 resulting from HHT treatment enhances the susceptibility of THP-1 cells to CD8+ T cell cytotoxicity, accompanied by increased markers of immune activation. Overall, our findings position HHT as a promising clinical agent for enhancing CD8+ T cell-based cancer immunotherapy by mitigating immune evasion in monocytic AML.

Background The prognosis of multiple myeloma (MM),has been improved by the emergence of novel agents such as immunomodulatory drugs and proteasome inhibitors, which have become the cornerstone of MM treatment. The persistent nature of MM and the relatively long survival of MM patients have raised concerns over the safety and efficacy of continuous long-term therapy. The all-oral regimen consisting of ixazomib(I), lenalidomide(R), and dexamethasone(D)(IRD) has been widely used in the treatment of myeloma, which greatly improves patient adherence and facilitates medication application; however, there is a lack of real-world clinical data as to whether IRD maintenance therapy improves patient's outcome. This study reports long-term real-world data from 59 myeloma patients who were switched to IRD maintenance therapy. Methods The median age of 59 patients with multiple myeloma was 66 years with 35 (59%)patients≥65 years. International Myeloma Working Group (IMWG) risk stratification was highrisk in 45 (76%)patients, and Intermediate risk in 14 (24%)patients. The amplification of 1q21 (1q21 amp) was found in 18 (31%) patients. Patients included in the observation group had received at least two courses of different induction therapies with treatment outcomes of stable disease(SD) or better were converted to an IRD all-oral regimen with weekly ixazomib 4mg or 3mg for 3 weeks, lenalidomide 25mg qd for 21d consecutively, and weekly dexamethasone 20mg orally for 3 weeks. The patients were evaluated for the remission rate (patients reached partial response[PR], very good partial response[VGPR], and complete response[CR]), duration of remission, and adverse events (AEs) after IRD treatment. The cutoff time was July 1, 2024. Results Fifty-nine patients were treated with the IRD regimen for a median of 25 months(12-55), with a maximum duration of 55 months. Twenty-two (37.3%) patients received oral therapy at the cutoff for follow-up. After six courses of treatment, 79.7% of patients reached (15 vs 47)VGPR, with 6 of 9 (66.7%)SD patients achieving VGPR or higher after switching to oral therapy and 15 of 24 (62.5%)PR patients achieving VGPR. Subgroup analyses showed that patients with 1q21 amp, either in the IMWG high-risk or Intermediate-risk group, could benefit from a treatment switch, with the VGPR rate increasing from 50% to 78% after switching to I-base oral regimens and increasing to 73% and 100% in high-risk and Intermediate-risk patients, respectively. In patients ≥65 years of age after switching to an oral I-base regimen, VGPR rates increased from 28.5% to 80% with a mean DOT of 8.2 months when evaluated over 6-12 courses. The ixazomib-based oral maintenance regimen was generally well-tolerated by patients. The major adverse effect of the bortezomib-based induction regimen was peripheral neuropathy (PN), which reached grade 3 or more in 7 patients. No TAEs above grade 3 occurred after switching treatments. The major adverse effect of the ixazomib-based oral maintenance regimen was diarrhea, which mostly disappeared after the intervention, with only one patient seeing a worsening of PN that led to termination of treatment. Discuss Clinical studies have shown that continuous maintenance therapy after induction therapy for MM improves treatment outcomes and prognosis, especially to patients at high cytogenetic risk. Real-world data from our center showed that maintenance therapy with IRD improved the depth of remission and reduced the risk of disease progression in high-risk patients. Although the induction therapy regimen before the study was different, 80% of the patients improved the depth of remission after long-term sustained IRD maintenance therapy, which provides an option for follow-up treatment of high-risk myeloma patients. Despite the limited cases in this study, we still noticed that the IRD regimen achieved significant therapeutic responses in elderly patients with comorbidities, regardless of age, gender, ISS stage, renal insufficiency, comorbidities status, first-line therapeutic exposures, or 1q21+status, which is similar to literature. The main adverse effect of the maintenance regimen was diarrhea. The longest maintenance treatment duration reached 55 months in this study, with no significant hematologic toxicity, PN exacerbation, or herpes zoster infection during treatment. Patient compliance and quality of life were improved.

Background Although the multi-drug chemotherapy regimen known as VDCLP can induce hematological remission in 70-90% of adults with acute lymphoblastic leukemia, many patients, particularly young women, remain concerned about chemotherapy-related complications such as alopecia, infection, and bleeding risk. Blinatumomab, a bispecific monoclonal antibody construct that enables CD3-positive T cells to recognize and eliminate CD19-positive acute lymphoblastic leukemia (ALL) blasts, has notably enhanced the remission rates for relapsed and refractory adult ALL when combined with chemotherapy. Additionally, the combination of blinatumomab and TKI has demonstrated significant efficacy in treating ph+ adult ALL. However, there is currently no clinical evidence indicating whether blinatumomab, as a first-line therapy, can achieve higher remission rates and reduce toxic and side effects in newly diagnosed adult ALL patients. In this report, we present the findings from our center where we have used the blinatumomab regimen as the primary treatment for adult ALL. Method A total of 12 newly diagnosed adult ALL patients were enrolled in this study. These patients consisted of 3 males and 9 females, with ages ranging from 23 to 72 years. All patients underwent bone marrow aspiration and were diagnosed according to morphology, immunophenotyping, cytogenetics, and molecular biology（MICM）. They were categorized into 7 cases of Ph+ ALL and 5 cases of Ph- ALL(Table1). All patients received a combination regimen consisting of vincristine (1.4mg/m2 on D1 and D8), prednisone (1mg/kg orally from D1-14,decrease gradually after D15), and blinatumomab. Blinatumomab was initiated on D3 and administered in an escalating dose manner for 15-28 days (9 μg/day from D1-7, 28 μg/day from D8) . Result Seven patients completed the 15-day treatment course, and five patients completed the full 28-day treatment course. Bone marrow morphology and MRD assessments were conducted on days 15 and 28 post-treatment. On day 15, the hematological remission rate (HCR) of bone marrow reached 100% (12 out of 12 patients), while the MRD negativity rate was 16.7% (2 out of 12 patients tested). five patients completed the full 28-day treatment course, and upon re-examination on day 28, maintained a 100% HCR. Among these, the MRD negativity rate improved to 80% (4 out of 5 patients). No significant difference in HCR was observed between ph+ ALL and ph- ALL groups. Five patients underwent allogeneic stem cell transplantation, and four of these patients were thriving, while one patient died of COVID-19 infection 3 months after transplantation. Of the seven patients without a transplant, two elderly patients received non-chemotherapy maintenance treatment but unfortunately passed away due to disease recurrence within 12 months. Four patients with ph+all received alternating chemotherapy and oral TKI treatment and are currently in good health and alive. One patient with ph-all received CAR-T therapy and still alive with MRD negative. (Figure1) Discuss Our center's findings further corroborate that for adults with new diagnosis Ph+/- ALL, initial treatment involving blinatumomab combined with low-dose chemotherapy can attain a 100% HCR rate, absent of pronounced toxicity or side effects, thereby offering a viable option for patients intolerant or averse to chemotherapy. Currently, there is insufficient evidence to determine the ideal duration for blinatumomab treatment in adult ALL patients. However, for refractory and relapsed ALL cases, a continuous 28-day course of blinatumomab therapy may be required. Initial findings from our center indicate that after 15 days of VP+blinatumomab therapy, all patients achieved hematological remission. This outcome is particularly advantageous for patients in developing nations and regions. Switching to chemotherapy or oral TKI promptly after 15 days of blinatumomab treatment can considerably decrease treatment expenses and minimize complications associated with combined chemotherapy. Nevertheless, the sample size in this study is limited, necessitating further clinical evidence to validate this perspective.

目的 观察优化急诊绿色通道护理模式对急性心肌梗死患者急救效率及不良心血管事件发生的影响.方法 选择2020年4月—2022年4月本院收治的74例急性心肌梗死(Acute myocardial infarction,AMI)患者作为研究对象,按照入院时间顺序分为对照组(2020年4月—2021年4月)与观察组(2021年5月—2022年4月)两组,每组各37例.对照组实施常规急诊绿色通道护理,观察组实施优化急诊绿色通道护理模式.比较两组救治时间、救治成功率、心功能改善、不良心血管事件发生情况.结果 观察组患者急诊出诊时间、现场急救时间、急诊至介入室或溶栓时间、抢救时间均短于对照组,差异有统计学意义(P<0.05);观察组患者救治成功率高于对照组,差异无统计学意义(P>0.05);两组患者救治前心功能分级比较,差异无统计学意义(P>0.05).两组患者救治后心功能均得到改善,且观察组患者心功能改善优于对照组,差异有统计学意义(P<0.05);观察组患者不良心血管事件发生率低于对照组,差异有统计学意义(P<0.05).结论 优化急诊绿色通道护理模式可缩短AMI患者急救时间,改善患者心功能,降低不良心血管事件发生率,提高救治成功率.

Background ： The 5-year overall survival rate (OS) for children with thalassemia major who undergo allogeneic stem cell transplantation is 90% and the event-free survival (EFS) rate is 86%. Due to the limited availability of matched sibling donors, the proportion of transplantations from alternative donors is increasing, particularly from haplo-identical donors. Post-transplant infections and graft-versus-host disease (GVHD) remain the major causes of mortality, especially in those who receive transplantation from alternative donors. Post-transplant cyclophosphamide (PTCY) has shown outstanding results of GVHD prophylaxis in haploidentical matched related donors transplantation with hematological malignancies. However, there is insufficient clinical evidence for its application in pediatric patients who received transplantation from haplo-identical donors with non-malignant hematological disease. Here, we retrospectively analyzed the results of 40 cases that underwent allogeneic stem cell transplantation from haploidentical matched related donors (Haplo-RD) and unrelated donors (UD) to evaluate the safety and efficacy of the novel regimen in GVHD prophylaxis. Method A total of 40 patients with β-thalassemia major received stem cell transplantation, 10 haploid donors (Group Haplo-RD), and 30 unrelated donors (Group UD). Among Group UD, 15 donors were 1 or 2 HLA locus mismatched, remaining 15 were identically matched. The median age of the patients was 5.5 years, ranging from 2 to 12. The Conditioning regimen mainly consisted of fludarabine, busulfan, cyclophosphamide, and thiotepa. GVHD prophylaxis included ATG 1.5mg/kg×3d, cyclophosphamide (CTX) 25mg/kg×2d, cyclosporin A (CsA), and mycophenolate mofetil (MMF). We analyzed the engraftment rate, GVHD incidence, survival rate, and virus reactivation rate for each group. Results Engraftment rate was 100%, with a median time of 11 (10-15) days for neutrophil implantation and 12 (6-31) days for platelet implantation. All patients achieved complete chimerism on day 30 after transplantation. Three cases (7.5%) suffered from Ⅲ-Ⅳ aGVHD, with one being in the Group Haplo-RD group and two in the Group UD. Four cases (10.0%) of cGVHD were recorded. The overall survival (OS) rate and thalassemia-free survival rate were both 92.5%. In Group Haplo-RD, both the OS and thalassemia-free survival rates were 100%. Eight cases reported cytomegaloviremia but no one developed to CMV disease. The total activation rate for CMV was 20%. Nine cases suffered from BK virus urinary tract infection, resulting in a total activation rate of 22.5%. Detailed results are shown in Table 1 and Figure 1. Discussion Due to the high risk of GVHD and infection, stem cell transplantation from haploidentical and unrelated donors cannot be routine therapy for children with thalassemia major. Standard-dose PTCY regimen significantly increases the risk of CMV and BK infection after transplantation and is not suitable for non-malignant disease. The results have been reported that low-dose PTCY/ ATG can reduce the risk of GVHD as compared with standard-dose ATG in haploidentical donor stem cell transplantation. In our study, the novel GVHD prophylaxis regimen, semi-dose PTCY (25mg/kg×2d) combined with low-dose ATG (1.5mg/kg×3d), was used for stem cell transplantation from haploidentical and unrelated mismatched donors. The incidence of III-IV aGVHD was 7.5% and cGVHD was 10.0%, and there was no rejection of the transplant. The activation rate of CMV and BKV was significantly lower than reported in the literature, and the non-relapse mortality (NRM) after transplantation was significantly reduced. There was no statistical difference in the rate of OS, EFS, aGVHD, and cGVHD between Group Haplo-RD and Group UD. The results provide an effective approach for preventing GVHD and infectious diseases in thalassemia patients who underwent allo-HSCT with high-risk GVHD.

Abstract Purpose:: Multiple myeloma(MM) is a common malignant tumor in the blood system. Despite recent advances in its treatment, its symptomatic remission rate and survival rate are still not optimistic. In the future, it is necessary to continue to search for different treatment targets and new treatment methods in order to improve the quality of life and survival time of patients with MM. The study aims to explore the potential immune related pivotal genes and immune infiltration patterns in MM. Methods: The study included peripheral blood samples from patients with MM who our hospital from October 2020 to April 2022. Obtain a gene chip for research from a comprehensive gene expression database, perform differential expression analysis on the processed gene dataset, and then perform functional enrichment analysis, weighted gene co expression network analysis, GSEA immune infiltration analysis, and LASSO regression analysis on the obtained differential expression genes to obtain the hub genes. Finally, the hub gene TNFSF14 (LIGHT) was validated by qRT-PCR. Results: In the study, three immune-related hub genes (ADAM8, CR2, and TNFSF14) and three main types of peripheral immune cells (activated CD8 T cells, macrophages, and plasma cell like dendritic cells) were obtained, which are closely related to the pathogenesis of MM. Then, by collecting peripheral blood samples from some patients in our hospital and conducting real-time fluorescence quantitative polymerase chain reaction, it was confirmed that the hub gene TNFSF14 (LIGHT) mined in this study was highly expressed in peripheral blood samples from patients with MM, which may indicate that it plays a pathogenic role in MM. Conclusion: The study found that immune-related hub genes (ADAM8, CR2, and TNFSF14) are closely related to the pathogenesis of MM, and should be further researched.

目的 分析输血依赖型地中海贫血儿童非亲缘造血干细胞移植后BK病毒(BKV)相关出血性膀胱炎的发生率、临床特征和影响因素.方法 回顾性分析2018 年2 月至2021 年2 月厦门大学附属中山医院62 例输血依赖型地中海贫血儿童接受非亲缘造血干细胞移植后的临床资料,包括患儿年龄、人类白细胞抗原(HLA)相合程度、急性移植物抗宿主病(aGVHD)和慢性移植物抗宿主病(cGVHD)的发生以及与BKV感染的关联性.结果 14 例(22.58%,14/62)发生出血性膀胱炎,尿液中均检出BKV感染,BKV copies最高达107/ml,出血性膀胱炎与BKV感染符合率为100.00%.在预处理方案相同情况下,非亲缘全相合、移植物抗宿主病(GVHD)是发生BKV相关出血性膀胱炎的影响因素.结论 BKV感染是输血依赖型地中海贫血儿童非亲缘造血干细胞移植后发生出血性膀胱炎的主要原因,非亲缘全相合、GVHD是发生BKV相关出血性膀胱炎的影响因素.

Objective To explore and analyze the value of nurse-led nursing for patients in emergency intensive care unit. Methods 80 patients treated in the emergency intensive care unit of the First Affiliated Hospital of Xiamen University from January 2020 to January 2021 were selected and randomly divided into control group and study group,with 40 cases each. The control group adopted routine care, and the study group applied the nurse-led care model based on the control group. Observed and compared the glucose control, insulin control dosage, glucose control stability and quality of life before and after care in the two groups. Results After nursing, the incidence of blood glucose level, Ave, SD, CV, GLI and hypoglycemia in the study group was lower than that of the control group, the time of blood glucose standard was shorter than that of the control group, the insulin dosage was less than that of the control group, and the quality of life score was higher than that of the control group, and the difference was statistically significant（P＜0.05）. Conclusion Nurse-led nursing mode has significant effect on blood glucose management of patients in emergency intensive care unit, which is of great significance in improving blood glucose, promoting blood glucose standards, reducing insulin volume, and reducing the incidence of hypoglycemia. At the same time, this nursing mode can ensure the stability of blood glucose control and make the whole process of hypoglycemia safer, with high clinical application value, worthy of popularization and application.

BackgroundTo evaluate the effect of addition of ruxolitinib in Graft-versus-Host Disease (GVHD) prophylaxis on pediatric patients with beta-thalassemia major after allogeneic hematopoietic stem cell transplantation(HSCT). MethodsThis retrospective study reviewed 49 consecutive beta-thalassemia major pediatric patients who underwent HSCT from unrelated or haploidentical donors from February 2018 to October 2022. All transplantation recipients received cyclosporine A (CsA), mycophenolate mofetil (MMF), and short-term methotrexate (MTX) as GVHD prophylaxis; while 27 of them in the ruxolitinib group had added ruxolitinib oral to GVHD prophylaxis regimen at 2.5 mg twice daily once successful engraftment after January 2020. ResultsThe outcome showed that the ruxolitinib group had a lower cumulative incidence than the control group regardless of acute GVHD (22.2% vs.40.9%; p = .153) or chronic GVHD (18.5% vs.40.9%; p = .072); especially, the incidence of grade III-IV acute GVHD was reported significantly less frequently in ruxolitinib group than that of the control group (0 vs. 27.3%, p = .005). No significant difference was detected between the two groups in EBV (Epstein-Barr virus)/CMV (cytomegalovirus) reactivation and BKV (BK virus) infection (p = .703, 1.000, and .436, respectively). Twenty-six patients (96.3%) in the ruxolitinib group were alive, while two patients (9.1%) in the control group died of intestinal acute GVHD. The 2-year overall survival (OS) and thalassemia-free survival (TFS) were both 96.296% in the ruxolitinib group, while both 90.909% in the control group. ConclusionThis study reveals that ruxolitinib prophylaxis is a promising option to decrease the incidence of grade III-IV acute GVHD in pediatric patients with beta-thalassemia major.

Abstract Purpose:: Multiple myeloma(MM) is a common malignant tumor in the blood system. Despite recent advances in its treatment, its symptomatic remission rate and survival rate are still not optimistic. In the future, it is necessary to continue to search for different treatment targets and new treatment methods in order to improve the quality of life and survival time of patients with MM. The study aims to explore the potential immune related pivotal genes and immune infiltration patterns in MM. Methods: The study included peripheral blood samples from patients with MM who our hospital from October 2020 to April 2022. Obtain a gene chip for research from a comprehensive gene expression database, perform differential expression analysis on the processed gene dataset, and then perform functional enrichment analysis, weighted gene co expression network analysis, GSEA immune infiltration analysis, and LASSO regression analysis on the obtained differential expression genes to obtain the hub genes. Finally, the hub gene TNFSF14 (LIGHT) was validated by qRT-PCR. Results: In the study, three immune-related hub genes (ADAM8, CR2, and TNFSF14) and three main types of peripheral immune cells (activated CD8 T cells, macrophages, and plasma cell like dendritic cells) were obtained, which are closely related to the pathogenesis of MM. Then, by collecting peripheral blood samples from some patients in our hospital and conducting real-time fluorescence quantitative polymerase chain reaction, it was confirmed that the hub gene TNFSF14 (LIGHT) mined in this study was highly expressed in peripheral blood samples from patients with MM, which may indicate that it plays a pathogenic role in MM. Conclusion: The study found that immune-related hub genes (ADAM8, CR2, and TNFSF14) are closely related to the pathogenesis of MM, and should be further researched.

目的:提高对异基因造血干细胞移植（allo-HSCT）后发生肺部移植后淋巴组织增殖性疾病（PTLD）的认识。方法:回顾性分析厦门大学附属中山医院2例allo-HSCT后肺部PTLD患者的临床资料，并复习相关文献。结果:2例患者均在移植后1年内诊断为肺部PTLD，伴EB病毒（EBV）血症。例1接受利妥昔单抗联合减停免疫制剂治疗，例2单纯减停免疫抑制剂治疗，2例患者均缓解，EBV-DNA均转阴性。结论:PTLD是HSCT后一种罕见的严重并发症，大多与EBV感染有关，累及肺部者罕见。对可疑PTLD患者需尽早行病灶穿刺活组织检查。

OBJECTIVE:To analyze the clinical efficacy of haploidentical hematopoietic stem cell transplantation (haplo-HSCT) by using parental donors on thalassemia patients.METHODS:The 13 thalassemia patients treated by haplo-HSCT using parental donors in our hospital from July 1, 2016, to July 1, 2020 were retrospectively reviewed. Hematopoiesis reconstitution, the incidence of GVHD, infections and the long-term survival of the patients were analyzed.RESULTS:Twelve of the 13 patients were successfully implanted, the success rate of implantation was 92.3%. The median time of neutrophil and platelet engraftment was 12.5 days (range, 9-22 days) and 21 days (range,12-34 days), respectively. One patient achieved primary graft failure. Three (25%) patients developed to acute GVHD (aGVHD) and achieved complete remission after treatment. Chronic GVHD developed in three (25%) patients, one of them was extensive and under treatment, while one patient developed to severe bacterial infection (7.7%). CMV viremia was diagnosed in two patients (15.4%). There were no patients developed to CMV disease. Three (23.1%) patients achieved EB viremia after transplantation, one of them developed to EBV-related lymphocytic proliferative disease, while there were no patients showed invasive fungal infection. At the last follow-up, all patients survived, twelve of them were free from transfusion dependency. There were no transplant-related deaths. Projected overall and thalassemia-free survival at three years was 100% and 92.3%, respectively.CONCLUSION:The transplant protocol of haplo-HSCT by using parental donors in patients with thalassemia has reliable source of donors, high incidence of successful implantation and low incidence of GVHD, which can be used as an effective way to increase the source of donors in children with thalassemia.

目的 分析标准急救护理流程用于ST段抬高型心梗(STEMI)抢救和预后改善中的作用.方法 回顾性分析2020年1月至2021年11月本院收入STEMI患者合计68例的临床资料,结合不同急救方式将其中34例归为对照组(依据常规提供急救护理),余下34例归为观察组(经标准急救护理流程提供急救护理),对比两组在急救指标、急救效果、焦虑及抑郁评分方面的差异性.结果 观察组静脉通路开放、心电图应用、抢救时间及住院时间均短于对照组(P＜0.05).观察组的抢救成功率高出对照组,复发率低于对照组(P＜0.05).护理前,两组焦虑和抑郁得分相比无差异(P＞0.05);护理后,观察组的焦虑和抑郁得分均低于对照组(P＜0.05).结论 标准急救护理流程用于STEMI患者能提升其抢救效率及效果,减轻其负面情绪,降低其复发率,改善其预后,值得采用.

目的 探讨融合抑制(SUFU)基因多态性与地中海贫血(TM)患儿接受异基因造血干细胞移植(allo-HSCT)后发生移植物抗宿主病(GVHD)之间的关系.方法 选择2018年10月1日至2020年12月31日厦门大学附属中山医院接收的44例接受allo-HSCT的TM患儿作为研究对象,经SUFU rs17114808位点基因多态性检测,比较不同基因型患儿的临床资料,记录GVHD的发生率和严重程度,并分析SUFU基因型与移植后发生GVHD的关系.结果 44例TM移植患儿中,SUFU基因位点(rs17114808)CC、CT、TT的基因型发生率分别为29.55％、52.27％、18.18％.CC型组和CT/TT型组性别、移植年龄、供受者关系、人类白细胞抗原匹配程度、末次随访存活率比较,差异无统计学意义(P>0.05).移植后CC型组急性GVHD(aGVHD)和慢性GVHD(cGVHD)的发生率均高于CT/TT型组,但差异无统计学意义(P>0.05).多因素logistic回归分析显示,携带CC基因型对Ⅱ～Ⅳ度aGVHD的发生有影响(OR=6.601,P<0.05).两组基因型cGVHD累及器官范围及受累器官类型比较,差异无统计学意义(P>0.05).结论 SUFU基因CC型可能与TM患儿移植后发生严重aGVHD有关,可作为预测发生Ⅱ～Ⅳ度aGVHD的指标.

Six children with steroid resistant graft versus host disease (GVHD) after hematopoietic stem cell transplantation admitted in the hospital, including 4 cases of acute GVHD and 2 cases of chronic GVHD. Among the 4 acute GVHD cases, the main manifestations were large area rash and fever in 2 cases, and abdominal pain and diarrhea in 2 cases. In 2 chronic GVHD cases, one presented lichenoid dermatosis, and the other showed repeated oral ulcers with difficult mouth opening. Patients received tocilizumab (8 mg/kg per dose every 3 weeks) and ruxolitinib (5-10 mg/d, 28 d), at least 2 courses were completed. All patients had complete responses (100%), and 5 patients responded after completion of two treatment courses, with the median time of remission was 26.7 d. The median follow-up period was 11 (7-25) months, and no severe treatment-related adverse reactions were observed.

Objective:To explore the efficacy and safety of unrelated mismatched hematopoietic stem cell transplantation(HSCT)for thalassemia major.Methods:For this retrospective cohort study, 15 patients with β-thalassemia major underwent unrelated mismatched HSCT between January 2018 and April 2022. There were 8 males and 7 females with a median age of 7(3-12)years and a median ferritin level of 3 417.3(223-14 485)μg/L. The conditioning regimens on the basis of fludarabine(Flu), busulfan(Bu)and cyclophosphamide(CTX)and GVHD prophylaxis on the basis of cyclosporine(CsA), mycophenolate mofetil(MMF), anti-human thymocyte immunoglobulin(ATG)plus low-dose post-cyclophosphamide(PTCy)and mesenchymal stem cells were offered.Results:Up until April 1, 2022, 15 children were successfully implanted during a median follow-up period of 24.1(11-49)months and all of them achieved stable donor chimerism. The median time to neutrophil and platelet engraftment were 12(11-22)and 14(8-38)days respectively. Except for 2 deaths, 13 cases survived. The estimated 2-year probability of overall survival(OS)and thalassemia-free survival(TFS)were both 86.67%. There were 5 cases of acute graft versus host disease (aGVHD) below grade Ⅱ, 2 cases of grade Ⅲ to Ⅳ aGVHD, and 3 cases of localized chronic graft versus host disease (cGVHD) after transplantation. No gengralized cGVHD occurred. Both cytomegalovirus and Epstein-Barr virus were activated in five recipients.Conclusions:Unrelated mismatched donor HSCT is both safe and feasible for thalassemia major.

Objective:To explore the safety and advantages of non-cryopreserved sibling umbilical cord blood hematopoietic stem cell transplantation for major thalassaemia in children.Methods:From October 2016 to June 2021, 9 patients with major beta thalassaemia received non-cryopreserved hematopoietic stem cell transplantation of sibling umbilical cord blood at Zhongshan Hospital of Xiamen University. The pretreatment scheme, the process of stem cell implantation and follow-up were analyzed and summarized.Results:Among the 9 cases, there were 5 males and 4 females with a median age of 4(2~11)years. Median level of ferritin was 2 997(1 936~5 512)μg/L. At gestational weeks 12~16, each patient's mother underwent villi testing to confirm that the donor without thalassaemia major was complete HLA-matched with the patient. All of them received an intensive conditioning regimen made up of cyclophosphamide(CTX), fludarabine and busulfan(Bu). Graft-versus-host disease(GVHD) was prevented by cyclosporine A(CSA)and mycophenolate mofetil(MMF)with or without methotrexate(MTX). Except for one failed implant, 8 cases were successfully engrafted. Median time of neutrophil implantation was 19.5(15~26)days, median time of platelet implantation 32(22~34)days and median time of erythrocyte implantation 30.5(18~37)days. Up until September 1, 2021, the median follow-up period was 27(3~59)months and the rate of successful engraftment 88.89%. There was no transplant-related mortality. Overall survival was 100% and thalassaemia-free survival 88.89%. Two patients developed grades Ⅱ skin acute GVHD(22.2%). No grade Ⅲ-Ⅳ GVHD or chronic GVHD occurred. Epstein-Barr virus infection occurred in 1 case.No infection of cytomegalovirus occurred.Conclusions:For major thalassaemia in children, stem cell transplantation of non-cryopreserved sibling cord blood is both safe and feasible with a high implantation rate and a low incidence of GVHD.

BACKGROUND BCR-ABL1 fusion gene is associated with a poor prognosis and a high incidence in central nervous system (CNS) leukemia. CNS invasion which detected at the initial diagnosis is commonly with bone marrow infiltration. It is uncommon for the leukemia cells to be located primarily in the CNS without bone marrow involvement. CASE SUMMARY We here report the rare initial presentation of CNS-restricted BCR-ABL-positive acute lymphoblastic leukemia in a 30-year-old female patient who clinically manifested with leukemic meningitis, with no involvement in peripheral blood or bone marrow. Identification of abnormal phenotypes of blast cells, and BCR-ABL1 rearrangement in the cerebrospinal fluid alone established the diagnosis of primary CNS-isolated acute lymphocytic leukemia. The patient received a combination of intrathecal therapy and high-dose chemotherapy. But the benefits of the treatments were short-lived and she experienced recurrence. CONCLUSION Flow cytometry in combination with molecular genetic analysis improved diagnostic accuracy. New approaches that may enhance the efficacy of the existing therapies and cure CNS leukemia are required.