Aimaz Afrough, Pearl Rajan Abraham, Laura Turer, Gurbakhash Kaur, Aishwarya Sannareddy, Doris K Hansen, Larry D Anderson
{"title":"Toxicity of CAR T-Cell Therapy for Multiple Myeloma.","authors":"Aimaz Afrough, Pearl Rajan Abraham, Laura Turer, Gurbakhash Kaur, Aishwarya Sannareddy, Doris K Hansen, Larry D Anderson","doi":"10.1159/000539134","DOIUrl":"10.1159/000539134","url":null,"abstract":"<p><strong>Background: </strong>Idecabtagene vicleucel (ide-cel) and ciltacabtagene autoleucel (cilta-cel) are novel chimeric antigen receptor (CAR)-T cell therapies targeting B-cell maturation antigen (BCMA), and both have recently gained approval by the US Food Drug Administration (FDA) for the treatment of relapsed and refractory multiple myeloma (RRMM).</p><p><strong>Summary: </strong>These therapies offer unprecedented responses in RRMM but present new challenges including cytokine release syndrome (CRS), immune effector cell-associated neurotoxicity syndrome (ICANS), non-ICANS neurotoxicity, cytopenias, infections, and hypogammaglobulinemia.</p><p><strong>Key messages: </strong>In the evolving CAR-T landscape, a primary objective is to develop innovative strategies for managing associated toxicities. Through meticulous exploration of underlying mechanisms and tailored interventions, we aim to enhance safety and enable broader outpatient utilization. Refinement of protocols, biomarker identification, and robust monitoring are imperative for sustained efficacy. This comprehensive approach guarantees the continuous advancement and optimization of CAR-T therapy.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"1-15"},"PeriodicalIF":2.4,"publicationDate":"2024-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140891042","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Historical Perspective of High-Dose Therapy Followed by Autologous Stem Cell Transplantation in Multiple Myeloma.","authors":"Inbar Cohen, Iuliana Vaxman, Morie A Gertz","doi":"10.1159/000539225","DOIUrl":"10.1159/000539225","url":null,"abstract":"<p><strong>Background: </strong>High-dose therapy (HDT) followed by autologous stem cell transplantation (ASCT) has become part of standard of care (SOC) in newly diagnosed multiple myeloma. In this review, we provide a historical perspective on ASCT since its introduction in the 1990s.</p><p><strong>Summary: </strong>Overall survival (OS) benefit for HDT followed by ASCT was demonstrated in studies comparing HDT with ASCT to standard-dose therapy (SDT) before the era of novel agents. Conditioning is done with melphalan 200 mg/m2. Lower doses (MEL140, MEL150) for older patients with comorbidities are safe and have comparable results. The addition of busulfan to melphalan improves progression-free survival (PFS) but not OS. HDT with ASCT after induction with novel agents prolongs PFS but not OS compared to SDT alone. The benefit is more evident in patients with high-risk cytogenetics. Mobilization can be achieved with granulocyte colony-stimulating factor alone, but is improved with the addition of chemotherapy. Plerixafor reduces mobilization failure and enables sufficient stem cell collection after induction with novel agents. ASCT is safe with a low rate of mortality (1%), and selected patients can be managed as outpatients.</p><p><strong>Key messages: </strong>HDT followed by ASCT remains part of SOC due to its PFS benefit and relatively low toxicity.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"1-10"},"PeriodicalIF":2.4,"publicationDate":"2024-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140846838","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Utility of echocardiogram in neutropenic patients with gram positive bacteremia - a retrospective study.","authors":"Neta Sternbach, Haim Ben-Zvi, Ofir Wolach, Moshe Yeshurun, Pia Raanani, Dafna Yahav, Liat Shargian","doi":"10.1159/000538449","DOIUrl":"https://doi.org/10.1159/000538449","url":null,"abstract":"<p><strong>Introduction: </strong>Hemato-oncology patients are vulnerable to bloodstream infections due to immunocompromised state and use of intravascular catheters. Data regarding risk of infective endocarditis (IE) among those with gram positive bacteremia is limited. We aimed to evaluate the incidence of IE among neutropenic hemato-oncology patients, and to explore the yield of echocardiogram in this population.</p><p><strong>Methods: </strong>we conducted a single retrospective study of all hospitalized hemato-oncology neutropenic patients with gram positive blood cultures between 2007 and 2021. Data regarding Patients' characteristics, blood cultures and echocardiogram was collected.</p><p><strong>Results: </strong>Study included 241 patients, with 283 isolates. Coagulase negative staphylococcus (CONS) were the most commonly isolates found, followed by streptococcus viridans. Trans thoracic echocardiography (TTE) was performed in 45% of patients overall, of which 5.8% had additional Trans esophageal echocardiogram (TEE). Only a single case of IE was identified; 47 y/o multiple myeloma patient with neutropenic fever, streptococcus viridans bacteremia, and stroke caused by septic emboli. TTE and TEE failed to demonstrate valvular pathology consistent with IE. Conclusion In our experience, the yield of echocardiogram in hemato-oncological neutropenic patients with bacteremia is extremely low, owing to reduced probability of IE in this population, and thus could be avoided in most cases.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140287964","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Long Non-coding RNA RP11-252C15.1 is a Potential Biomarker of Prognosis and Hallmark for Leukemogenesis in Children with B-cell Precursor Acute Lymphoblastic Leukemia.","authors":"Lili Pan, Yongzhi Zheng, Hao Zheng","doi":"10.1159/000535461","DOIUrl":"https://doi.org/10.1159/000535461","url":null,"abstract":"<p><strong>Introduction: </strong>Improved understanding of the prognostic biomarkers associated with childhood acute lymphoblastic leukemia (ALL) is needed for accurate risk group stratification. This study aimed to identify potential long-non coding RNA (lncRNA) markers and evaluate their prognostic value in children with ALL.</p><p><strong>Methods: </strong>We selected 50 children with newly diagnosed ALL and 20 age-matched patients with idiopathic immune thrombocytopenia (controls). RNA sequencing was performed to identify differentially expressed lncRNAs between the ALL and control groups. Correlation analysis was performed to determine the relationships between candidate lncRNAs, clinical features, and the risk of leukemogenesis.</p><p><strong>Results: </strong>A total of 1,019 differentially expressed lncRNAs were identified between the ALL and control groups. Reverse-transcriptase (RT-qPCR) revealed that lncRNA RP11-252C15.1 and lncRNA RP11-701P16.2 were significantly upregulated in patients with ALL. Furthermore correlation analysis showed that lncRNA RP11-252C15.1 and lncRNA RP11-701P16.2 represent potential predictors of leukemogenesis; however, only lncRNA RP11-252C15.1 was associated with clinical features and outcome in children with B-cell precursor ALL (BCP-ALL). In vitro experiments confirmed that lncRNA RP11-252C15.1 was significantly overexpressed in BCP-ALL cell lines and promoted proliferation, and repressed apoptosis in MHH-CALL-3 cells.</p><p><strong>Conclusion: </strong>LncRNA RP11-252C15.1 is a potential oncogene in BCP-ALL pathogenesis and a prognostic biomarker in children with BCP-ALL.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140287962","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The ratio of serum urea nitrogen to albumin is a better predictor of OS in MM patients than urea nitrogen alone.","authors":"Jiaqi Shao, Enfan Zhang, Haoguang Chen, Zhen Cai, Mengmeng Dong","doi":"10.1159/000538479","DOIUrl":"https://doi.org/10.1159/000538479","url":null,"abstract":"<p><p>Introduction Multiple myeloma (MM) is a malignant proliferative disease of plasma cells. Abnormally cloned plasma cells secrete large amounts of monoclonal immunoglobulins in the bone marrow of MM patients. Serum urea nitrogen (sUN) is a byproduct of protein metabolism, and its effect on MM patients' prognoses remains unknown. Therefore, we analysed MM patients' clinical data to explore the role of sUN and serum urea nitrogen/serum albumin (sUAR) in the baseline tumor load and MM prognosis of MM patients. Methods We downloaded the clinical data of 762 MM patients from the MMRF database. After excluding those without baseline sUN, 452 patients were finally included in the study. Smoothed curve fitting, threshold analysis, Tamhane's T2 test, multivariate adjusted Cox regression analysis, Kaplan‒Meier (K-M) curves, and receiver operating characteristic (ROC) analysis were applied in the study. Results There were 452 newly diagnosed MM patients included in this study. In most patient groups, sUN and sUAR were positively linked with β2-microglobulin (β2-MG) and lactic dehydrogenase (LDH) according to smoothing curve fitting and threshold analysis. The higher the ISS stage, the greater the values of sUN and sUAR. Furthermore, smoothed curve fitting and threshold analysis showed that sUN was correlated with OS, although sUAR had a stronger correlation with OS and could be applied to a broader group. The results of a multivariate adjusted Cox regression analysis demonstrated that sUN and sUAR were independent prognostic factors for OS. The K-M curve confirmed the correlation between higher sUN and sUAR levels and worse OS. β2-MG and LDH are generally recognized prognostic factors of OS. ROC analysis revealed that sUN might boost β2-MG and LDH's predictive value and sUAR had a higher predictive value. Conclusion This retrospective study based on the MMRF database showed that high sUN and sUAR levels were positively associated with β2-MG, LDH, and ISS staging, and sUAR exhibited a stronger correlation with OS than sUN alone.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140287963","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Karolina Bednarska, Gayathri Thillaiyampalam, Sally Mujaj, Jamie Nourse, Jay Gunawardana, Muhammed B Sabdia, Soi C Law, Anna Pilaar, Qingyan Cui, Lilia M de Long, Frank Vari, Maher K Gandhi, Alexandre S Cristino
{"title":"The IRE1α-endonuclease plays a dual role in regulating the XBP1/miRNA-34a axis and PD-1 expression within Natural Killer cells in Hodgkin Lymphoma.","authors":"Karolina Bednarska, Gayathri Thillaiyampalam, Sally Mujaj, Jamie Nourse, Jay Gunawardana, Muhammed B Sabdia, Soi C Law, Anna Pilaar, Qingyan Cui, Lilia M de Long, Frank Vari, Maher K Gandhi, Alexandre S Cristino","doi":"10.1159/000536044","DOIUrl":"https://doi.org/10.1159/000536044","url":null,"abstract":"<p><strong>Introduction: </strong>Hodgkin Lymphoma (HL) is deficient in Major Histocompatibility Complex-class I, rendering it susceptible to anti-tumoral immunity by Natural Killer (NK)-cells. Despite the functional impairment of PD-1+ NK-cells in HL, the underlying mechanisms of NK-cell dysfunction remain unclear.</p><p><strong>Methods: </strong>This study involved 14 HL patients and SNK10/KHYG-1 cell lines to assess NK-cell activation against cancer cells. Activation was measured through transcript (PCR) and protein expression (flow cytometry). Regulatory mechanisms associated with IRE1α activation were validated through knock-down and luciferase reporter assays.</p><p><strong>Results: </strong>Our findings reveal a novel role for IRE1α-endonuclease in fine-tuning NK-cell effector functions by orchestrating the XBP1s/microRNA-34a-5p/PD-1 axis. When NK-cells encounter cancer cells, IRE1α-endonuclease activates the decay of microRNA-34a-5p, resulting in increased expression of XBP1s and PD-1. IRE1α-endonuclease activation enhances NK-cells function while promoting PD-1 expression. In turn, PD-1 is directly regulated by microRNA-34a-5p, which binds to the 3'UTR of PD-1 transcript to repress PD-1 protein on the NK-cell surface. Importantly, IRE1α-pathway activation is impaired in NK-cells from HL patients.</p><p><strong>Conclusion: </strong>The IRE1α-endonuclease emerges as a key player, simultaneously regulating the XBP1s/microRNA-34a-5p/PD-1 axis in NK-cells, a process disrupted in HL. Targeting the IRE1α-pathway holds promise as a therapeutic strategy to optimise NK-cell functions in Hodgkin Lymphoma treatments.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-03-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140118451","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ohad Benjamini, Tamar Tadmor, Abraham Avigdor, Rotem Gershon, Limor Kliker, Florin Fares, Nofar Atari, Ilana Laevsky, Bayan Abd Elkader, Tammy Hod, Orit Golan-Shany, Michal Mandelboim, Galia Rahav
{"title":"Efficacy of preexposure prophylaxis with monoclonal-antibody tixagevimab-cilgavimab against emerging SARS-CoV-2 resistant variants in patients with chronic lymphocytic leukemia.","authors":"Ohad Benjamini, Tamar Tadmor, Abraham Avigdor, Rotem Gershon, Limor Kliker, Florin Fares, Nofar Atari, Ilana Laevsky, Bayan Abd Elkader, Tammy Hod, Orit Golan-Shany, Michal Mandelboim, Galia Rahav","doi":"10.1159/000537690","DOIUrl":"https://doi.org/10.1159/000537690","url":null,"abstract":"<p><p>Introduction Pre exposure prophylaxis with monoclonal antibodies (mAbs) were developed in addition to COVID19 vaccine for immunocompromised and those with insufficient immune response, among them patients with CLL. Omicron variant and its sublineages evolved mutations that escape mAbs neutralizing effect, yet the extent of which was not studied. Methods We evaluated anti-spike titters and neutralization activity of COVID-19 wild type (WT) , Delta , Omicron, BA2, BA4 and BA5 before and after tixagevimab-cilgavimab (TGM/CGM) dose of 150/150mg or 300/300mg in patients with CLL. Results 70 patients were tested 2 weeks before and 4 weeks after receiving TGM/CGM mAbs. After TGM/CGM anti-spike ab level increased 170 folds from 13.6 BAU/ml (IQR, 0.4-288) to 2328 BAU/ml (IQR, 1681-3500). Neutralization activity increased in all variants, and was 176 folds higher in WT and 55 folds higher in Delta compared to 10 folds higher in Omicron and its sublineages (BA2 x11, BA4 x4 , BA5 x18). Over follow-up period of 3 months, 20 patients (29%) with CLL acquired COVID-19 infection, all recovered uneventfully. In a multivariate analysis anti-spike antibody titer was found a significant predictor for post TGM/CGM COVID19 infection. Conclusion Efficacy of preexposure prophylaxis with TGM/CGM in patients with CLL is significantly reduced in era of Omicron and its sublineages BA2, BA4 and BA5.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140108702","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Shirley Shapira, Naama Hirschberger, Yishai Ofran, Barak Mizrahi, Esti Mandel, Ohad Benjamini, Noa Rabinowicz, Adi Zoref-Lorenz
{"title":"The effect of mind-body intervention on lymphocyte doubling time and treatment free survival in treatment-naïve chronic lymphocytic leukemia patients.","authors":"Shirley Shapira, Naama Hirschberger, Yishai Ofran, Barak Mizrahi, Esti Mandel, Ohad Benjamini, Noa Rabinowicz, Adi Zoref-Lorenz","doi":"10.1159/000538055","DOIUrl":"https://doi.org/10.1159/000538055","url":null,"abstract":"<p><strong>Introduction: </strong>Mind-Body Intervention (MBI) serves as supportive aid in oncology. We hypothesized that MBI could impact the progression of Chronic Lymphocytic Leukemia (CLL) in the 'watch and wait' (w&w) phase.</p><p><strong>Methods: </strong>MBI was utilized in a non-randomized prospective controlled study between 02/2020-02/2022 in 76 treatment-naïve CLL patients in the w&w phase (37 intervention and 39 control patients). The primary and secondary endpoints were prolongation of Lymphocyte Doubling Time (LDT) and treatment-free survival (TFS). The prolongation of LDT was compared at 0, 180, 360, and 540 days using paired t-tests. TFS was compared between intervention and control groups using the log-rank test. Cox proportional hazards models were used to calculate the hazard ratio (HR) and 95% confidence interval (CI) for TFS in the intervention group compared to the control, stratified by the study covariates.</p><p><strong>Results: </strong>MBI prolonged LDT at all time points, including at day 360 (Median of 2.47 years; CI 1.05-3.9; p= 0.001). TFS at 18 months was longer in the intervention group compared to the control group (HR 0.23; CI 0.06-0.79, p=0.01).</p><p><strong>Conclusions: </strong>MBI was associated with prolonged LDT and TFS in patients with CLL in the w&w phase. These results provide a basis for a larger randomized-control trial.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-03-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140093268","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alexander D Heini, Vera Ulrike Bacher, Dilara Akhoundova, Katja Seipel, Thomas Pabst
{"title":"Peeling Back the Layers: Recurrent Talquetamab Skin Toxicity After Supportive Stem Cell Boost in Multiple Myeloma.","authors":"Alexander D Heini, Vera Ulrike Bacher, Dilara Akhoundova, Katja Seipel, Thomas Pabst","doi":"10.1159/000538047","DOIUrl":"https://doi.org/10.1159/000538047","url":null,"abstract":"<p><p>Bispecific antibodies have meaningfully expanded the therapeutic armentarium in multiple myeloma. Talquetamab is a CD3+ T cell redirecting antibody targeting GPRC5D, which is expressed on multiple myeloma plasma cells as well as in keratinized tissues. Due to the expression pattern, toxicity of talquetamab involves skin toxicity. Here we report the case of a patient who was treated with talquetamab after relapse after CAR-T therapy. The patient developed a severe recurrence of talquetamab-mediated skin toxicity after the administration of a supportive hematopoietic stem cell boost to treat persistent late cytopenias after CAR-T therapy. This case underscores the complex dynamics between novel immunotherapies like talquetamab and stem cell-based interventions in the context of MM treatment, shedding light on the need for personalized approaches to maximize the benefits of these therapies while minimizing their associated adverse effects.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-03-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140048505","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Dae-Ho Choi, Sang Eun Yoon, Junhun Cho, Seok Jin Kim, Won Seog Kim
{"title":"Primary nodal Epstein-Barr virus-positive T-cell/NK-cell lymphoma: Real-world experience.","authors":"Dae-Ho Choi, Sang Eun Yoon, Junhun Cho, Seok Jin Kim, Won Seog Kim","doi":"10.1159/000537962","DOIUrl":"https://doi.org/10.1159/000537962","url":null,"abstract":"<p><p>PTCL-EBV is a disease entity newly recognized in the WHO-HAEMS5 and the ICC of Mature Lymphoid neoplasms classification. Previously, it was classified as a subtype within PTCL-NOS and was known to have a poor prognosis. However, the clinical feature and treatment outcomes are not well known. This retrospective observational study was conducted on patients diagnosed with PTCL-EBV at Samsung Medical Center through a pathology review from 2000 to 2020. We analyzed clinical data from 14 patients. We conducted an investigation of patients with PTCL-EBV into immunohistochemistry and analysis of survival outcomes for each treatment regimen. We analyzed both overall survival and progression-free survival for each treatment regimen. 25% were beta-F1 positive, and 67% were TCRγ positive. TIA-1 and granzyme B exhibited positive results in all cases, whereas the NK cell marker CD56 was negative in only 11% of patients. The CD3 was observed in all of patients. And, the CD4 was 43% positive. The CD8 were investigated in 8 patients, with 37.5% positive. Hepatosplenomegaly was observed in 55% of patients, and 70% of patients displayed B symptoms at the time of diagnosis. Patients who received CHOP or CVP treatment had a median PFS of 2.2 months (95% CI 1.9-2.5 months), and patients who received other treatments had a median PFS of 5.1 months (NA). The objective response rate (ORR) for ICE/dexa as the first or second line treatment was 100% (3 out of 3). But, ORR of CHOP or CVP as the first line treatment was 33.3% (3 out of 9). The median overall survival (OS) for the group that received HSCT after achieving a response was 34.6 months (95% CI 0-74.6 months), and the median OS for the group that did not receive HSCT was 5.0 months (95% CI 2.1-7.9 months) (p=0.04). In conclusion, in the context of PTCL-EBV, despite a limited sample size, the ICE/Dexa regimen shows potential benefits in terms of ORR and PFS. Furthermore, the application of HSCT following the attainment of a complete response may prove advantageous.</p>","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140020713","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}