Current Research in Translational Medicine最新文献

{"title":"Effect of AML-exosomes on the cellular and molecular properties of bone marrow mesenchymal stromal cells: Expression of JAK/STAT signaling genes","authors":"Maryam Nabigol,&nbsp;Laya Khodayi Hajipirloo,&nbsp;Bentolhoda Kuhestani-dehaghi,&nbsp;Mehdi Allahbakhshian Farsani","doi":"10.1016/j.retram.2024.103474","DOIUrl":"10.1016/j.retram.2024.103474","url":null,"abstract":"<div><h3>Purpose of study</h3><div>Despite the various therapeutic options introduced for AML treatment, therapy resistance and relapse are still the main obstacles. It is well known that alterations in the bone marrow microenvironment (BMM) play a crucial role in leukemia growth and the treatment failure of AML. Evidence shows that exosomes alter the components of BMM in a way that support leukemia survival, leading to chemoresistance. In this study, we evaluated the effect of AML exosomes on the biological functions of human bone marrow mesenchymal stromal cells (h BM-MSCs), especially alteration in the expression of the JAK/STAT signaling genes, as a leukemia-favoring pathway.</div></div><div><h3>Method</h3><div>Exosomes were isolated from the HL-60 cell line and characterized using flow cytometry, Transmission Electron Microscopy (TEM), and Dynamic Light Scattering (DLS) technique. The exosome protein content was assessed using a bicinchoninic acid (BCA) protein assay kit in order to determine the concentration of exosomes. Subsequently, MSCs were treated with varying concentrations of AML exosomes, and data was obtained using MTT, cell cycle, apoptosis, and ki67 assays. Additionally, gene expression analysis was conducted through qRT-PCR.</div></div><div><h3>Result</h3><div>AML exosomes regulated the viability and survival of MSCs in a concentration-dependent manner. The qRT-PCR data revealed that treatment with AML exosomes at a concentration of 50 μg/mL led to a significant upregulation of JAK2, STAT3, and STAT5 genes in MSCs.</div></div><div><h3>Conclusion</h3><div>Because the JAK/STAT signaling pathway has been shown to play a role in the proliferation and survival of leukemic cells, our results suggest that AML exosomes stimulate MSCs to activate this pathway. This activation may impede AML cell apoptosis, potentially leading to chemoresistance and relapse.</div></div>","PeriodicalId":54260,"journal":{"name":"Current Research in Translational Medicine","volume":"73 1","pages":"Article 103474"},"PeriodicalIF":3.2,"publicationDate":"2024-09-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142376302","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 transplantation effect of pegylated granulocyte colony-stimulating factor mobilized hematopoietic stem cells may be superior to that of G-CSF mobilized hematopoietic stem cells in haploidentical allogeneic hematopoietic cell transplantation","authors":"Junjie Cao ,&nbsp;Xianxu Zhuang ,&nbsp;Renzhi Pei ,&nbsp;Ying Lu ,&nbsp;Peipei Ye ,&nbsp;Dong Chen ,&nbsp;Xiaohong Du ,&nbsp;Shuangyue Li ,&nbsp;Xuhui Liu","doi":"10.1016/j.retram.2024.103473","DOIUrl":"10.1016/j.retram.2024.103473","url":null,"abstract":"<div><div>Granulocyte colony-stimulating factor (G-CSF) mobilized peripheral blood stem cells have become the preferred source of hematopoietic stem cells. We compared the effectiveness of G-CSF and pegylated G-CSF (peg-G-CSF) for hematopoietic stem cell mobilization in haploidentical hematopoietic stem cell transplantation (haplo-HSCT) donors, and evaluated the transplant outcomes. We conducted a matched retrospective cohort study. Donors mobilized with peg-G-CSF (<em>n</em> = 70) and G-CSF (<em>n</em> = 70). 140 consecutive patients diagnosed with acute leukemia who underwent haplo-HSCT were included in this study. The findings revealed that the peg-G-CSF cohort exhibited significantly elevated myeloid-derived suppressor cells (MDSCs) levels in their grafts when compared to the G-CSF cohort (<em>P</em> &lt; 0.001). The 100-day cumulative incidence (CI) of grade III-IV acute graft-versus-host disease (GVHD) and 1-year CI of moderate-to-severe chronic GVHD were 4.3% vs 14.3 % (<em>P</em> = 0.047) and 11.2% vs 27.4 % (<em>P</em> = 0.023), in the peg-G-CSF group and G-CSF group. Patients reveiving mobilized stem cell with peg-G-CSF had a significantly greater likelihood of 1-year GVHD-free relapse-free survival (GRFS) compared to patients reveiving mobilized stem cell with G-CSF (74.9% vs 37.9 %, <em>P</em> &lt; 0.001). The higher graft MDSCs proportion was associated with lower grade II-IV aGVHD, cGVHD (<em>P</em> &lt; 0.05) and higher GRFS in the univariate analysis (<em>P</em> &lt; 0.05). Multivariate analysis showed that MDSCs proportion higher than 11.36 % (HR, 0.305; 95 % CI, 0.154–0.606; <em>P</em> = 0.001) and peg-G-CSF for stem cell mobilization (HR, 0.466; 95 % CI, 0.251–0.865; <em>P</em> = 0.016) were independent prognostic factors of GRFS. The superior survival rates observed in recipients of peg-G-CSF-mobilized cells are likely due to reduced acute GVHD, potentially mediated by the increased MDSCs within the grafts.</div></div>","PeriodicalId":54260,"journal":{"name":"Current Research in Translational Medicine","volume":"72 4","pages":"Article 103473"},"PeriodicalIF":3.2,"publicationDate":"2024-09-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142318689","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":"Point of care CD19 chimeric antigen receptor (CAR) T-cells for relapsed/refractory acute myeloid leukemia (AML) with aberrant CD19 antigen expression","authors":"Ivetta Danylesko ,&nbsp;Noga Shem-Tov ,&nbsp;Ronit Yerushalmi ,&nbsp;Elad Jacoby ,&nbsp;Amos Toren ,&nbsp;Roni Shouval ,&nbsp;Orit Itzhaki ,&nbsp;Abraham Avigdor ,&nbsp;Avichai Shimoni ,&nbsp;Arnon Nagler","doi":"10.1016/j.retram.2024.103471","DOIUrl":"10.1016/j.retram.2024.103471","url":null,"abstract":"<div><div>Relapsed/refractory (r/r) acute myeloid leukemia (AML) is associated with poor prognosis. CD19 is a B-cell marker, is aberrantly expressed in AML, mostly with t(8; 21)(q22; q22.1). Here we report the results of a phase 2 study giving point of care produced CD19 CAR T- cells for r/r AML with aberrant expression of CD19 (NCT04257175). Lymphodepletion included fludarabine and cyclophosphamide The response was evaluated by bone marrow (BM) aspiration on day 28. Six patients (5 adults and 1 child) were included. Median number of previous chemotherapy lines was 4 (range, 3–8) and four patients received CAR T-cells 8–18 months post allogeneic hematopoietic stem cell transplantation (allo-HSCT). Cytokine release syndrome (CRS) of any grade occurred in all patients, and 1 patient had grade 3 CRS. Immune effector cell-associated neurotoxicity syndrome (ICANS) occurred in 2 patients at low grades. Tocilizumab was administered to 2 patients and corticosteroids to 3 patients. Four patients achieved a complete remission (CR), while 2/6 progressed (PD). Three patients (2 with CR and 1 with PD) underwent allo-HSCT (it was the second transplant in 2) 2–5 months post CAR T-cells infusion. The median duration of response in patients achieving CR was 8.5 (range; 3–14) months. However, all patients eventually died within 5 (1–18) months.</div><div>In conclusion, CD19 CAR T- cell treatment for AML is feasible and safe. However, the response is short and should be followed by allo-HSCT. Hopefully, future long term results will be improved by combining the CAR T- cell therapy with the emerging novel effective anti-leukemic compounds.</div></div>","PeriodicalId":54260,"journal":{"name":"Current Research in Translational Medicine","volume":"72 4","pages":"Article 103471"},"PeriodicalIF":3.2,"publicationDate":"2024-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142300836","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":"Impaired survival of patients with non donor-specific anti-HLA antibodies before HLA-mismatched allogeneic stem cell transplantation","authors":"Antonio Milano ,&nbsp;Giuliana Lando ,&nbsp;Giulia Di Maggio ,&nbsp;Giorgia Cornacchini ,&nbsp;Giovanni Grillo ,&nbsp;Roberto Cairoli ,&nbsp;Silvano Rossini ,&nbsp;Roberto Crocchiolo","doi":"10.1016/j.retram.2024.103464","DOIUrl":"10.1016/j.retram.2024.103464","url":null,"abstract":"<div><h3>Background</h3><p>While the detrimental role of donor-specific anti-HLA antibodies (DSAs) is well-described in the setting of hematopoietic stem cell transplantation (HSCT), few studies focus on non donor-specific ones and with controversial results.</p></div><div><h3>Methods</h3><p>We here report our monocenter experience on 64 adult patients receiving allogeneic HSCT from a HLA-mismatched donor between 2014 and 2022 who were tested for the presence of anti-HLA antibodies before transplant, focusing on fifteen patients with non donor-specific anti-HLA antibodies.</p></div><div><h3>Results</h3><p>The survival of patients with non donor-specific anti-HLA antibodies was inferior with respect to patients without anti-HLA antibodies and similar to patients with DSAs. Median survival of patients with non donor-specific anti-HLA antibodies was 21 months (95 % CI: 9–42) vs. 61 months (95 % CI: 17–77) among the anti-HLA antibody-negative patients, with a significantly higher mortality incidence rate ratio (3.3 times-fold greater, <em>p</em> = 0.01). No pattern of death causes was found</p></div><div><h3>Conclusions</h3><p>In this monocenter series of HLA-mismatched HSCTs, impaired survival was observed in adult patients having non donor-specific anti-HLA antibodies before transplant, similar to those with DSAs. Our findings support those antibodies as a negative predictive factor even if they are not directed against the donor, thus warranting further investigation on larger cohorts.</p></div>","PeriodicalId":54260,"journal":{"name":"Current Research in Translational Medicine","volume":"72 3","pages":"Article 103464"},"PeriodicalIF":3.2,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142128416","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":"Feasibility of co-transplantation of umbilical cord blood and third-party mesenchymal stromal cells after (non)myeloablative conditioning in patients with hematological malignancies","authors":"Simon Planken ,&nbsp;Ann De Becker ,&nbsp;Tessa Kerre ,&nbsp;Hélène Schoemans ,&nbsp;Frédéric Baron ,&nbsp;Carlos Graux ,&nbsp;Ivan Van Riet ,&nbsp;Chantal Lechanteur ,&nbsp;Etienne Baudoux ,&nbsp;Rik Schots ,&nbsp;Yves Beguin ,&nbsp;Transplant Committee of the Belgian Hematology Society.","doi":"10.1016/j.retram.2024.103466","DOIUrl":"10.1016/j.retram.2024.103466","url":null,"abstract":"<div><p>Umbilical cord blood (UCB) is an alternative source of stem cells for patients lacking a 9/10 or 10/10 HLA identical donor. However, after UCB transplantation, time to engraftment and immune recovery are prolonged, increasing the risk of fatal complications. Mesenchymal stromal cells (MSC) can support hematopoietic engraftment and have immunosuppressive effects.</p><p>The primary objective of this phase I/II multicenter study was to determine the feasibility and safety of UCB transplantation with co-infusion of third party MSC, as assessed by treatment related mortality (TRM) at day 100. Secondary objectives were engraftment, immune recovery, occurrence of graft versus host disease (GVHD), infections, disease free survival, relapse incidence and overall survival.</p><p>Eleven patients were grafted according to this protocol. Allogeneic transplantation after co-infusion appears feasible with 18 % TRM at day 100. Engraftment data show a median time of 16 days to neutrophil and 27 days to platelet recovery, which is shorter than what is usually reported after UCB transplantation. Only 1 episode of acute GVHD was reported.</p><p>In conclusion, MSC and UCB co-transplantation is feasible and might help overcome some of the drawbacks of UCB transplantation.</p></div>","PeriodicalId":54260,"journal":{"name":"Current Research in Translational Medicine","volume":"72 4","pages":"Article 103466"},"PeriodicalIF":3.2,"publicationDate":"2024-08-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142098238","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":"Selective red blood cell depletion by apheresis in sheep causes severe normovolemic anemia","authors":"Anna-Lena Semmler ,&nbsp;Jan-Philipp Köhler ,&nbsp;Lara Regolien ,&nbsp;Franziska Bayer ,&nbsp;Nikolay Polyansky ,&nbsp;Elise Gradhand ,&nbsp;Andreas von Knethen ,&nbsp;Kai Zacharowski ,&nbsp;Fabian Nocke ,&nbsp;Katja B. Ferenz ,&nbsp;Joachim Schwäble ,&nbsp;Halvard Bonig ,&nbsp;Eva Herrmann ,&nbsp;Isabelle Hornung ,&nbsp;Andrea U. Steinbicker","doi":"10.1016/j.retram.2024.103463","DOIUrl":"10.1016/j.retram.2024.103463","url":null,"abstract":"<div><h3>Background</h3><p>: The setting of normovolemic anemia is required for a variety of research applications, such as testing of novel medication for anemia treatment. Unfortunately, large animal models using full blood draw and replenishment with balanced electrolyte solution (BES) lead to bleeding complications, as coagulation factors and platelets are also drawn. We therefore aimed to establish a model of selective red blood cell (RBC) depletion to the main endpoint of hemoglobin (Hgb) levels of 4–6 g dL⁻¹ using apheresis in sheep.</p></div><div><h3>Methods</h3><p>: <em>In vitro</em> experiments were performed first to establish the apheresis protocol. <em>In vivo</em>, anesthetized ewes underwent a sham protocol without apheresis (<em>n</em> = 5) or apheresis (<em>n</em> = 4). Both groups were observed for the following six hours at a defined starting point (BE0) to compare Hgb, hematocrit (Hct), coagulation and clinical parameters. For statistical analysis, unpaired <em>t</em>-test with Welch`s correction was used.</p></div><div><h3>Results</h3><p>: Hgb levels were effectively decreased by 51 % to mean Hgb of 4.4 g dL⁻¹ in the apheresis group compared to 9.1 g dL⁻¹ in sham (*<em>p</em> &lt; 0.0001). Hct (11.2% vs 25.1 %, *<em>p</em> = 0.01) and RBCs (3.7 vs 8.2 × 10⁶/µl, *<em>p</em> = 0.003) also decreased. The relative number of platelets compared to baseline was different (55.6 ± 10.6% vs. 100 ± 0 %, *<em>p</em> = 0.004), but no hemorrhage was observed. White blood cells (WBCs), lactate, prothrombin ratio and activated partial thromboplastin time (aPTT) remained within similar ranges.</p></div><div><h3>Conclusions</h3><p>: Critical normovolemic anemia without bleeding complications was successfully reached by selective RBC depletion in sheep. Investigations of physiological adaptations to severe anemia and pharmaceutical testing can be performed in large animals with depleted RBCs.</p></div>","PeriodicalId":54260,"journal":{"name":"Current Research in Translational Medicine","volume":"72 4","pages":"Article 103463"},"PeriodicalIF":3.2,"publicationDate":"2024-08-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142233057","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":"Clinical image: A new category of articles in current research in translational medicine","authors":"Tamim Alsuliman","doi":"10.1016/j.retram.2024.103462","DOIUrl":"10.1016/j.retram.2024.103462","url":null,"abstract":"","PeriodicalId":54260,"journal":{"name":"Current Research in Translational Medicine","volume":"72 3","pages":"Article 103462"},"PeriodicalIF":3.2,"publicationDate":"2024-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141772079","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":"Pitfalls in definitions on respiratory viruses and particularities of Adenovirus infection in hematopoietic cell transplantation patients: Recommendations from the EBMT practice harmonization and guidelines committee","authors":"José Luis Piñana ,&nbsp;Simone Cesaro ,&nbsp;Malgorzata Mikulska ,&nbsp;Paul E. Verweij ,&nbsp;Anne Bergeron ,&nbsp;Dionysios Neofytos ,&nbsp;Jan Styczynski ,&nbsp;Isabel Sánchez-Ortega ,&nbsp;Raffaella Greco ,&nbsp;Francesco Onida ,&nbsp;Ibrahim Yakoub-Agha ,&nbsp;Dina Averbuch ,&nbsp;Rafael de la Cámara ,&nbsp;Per Ljungman","doi":"10.1016/j.retram.2024.103461","DOIUrl":"10.1016/j.retram.2024.103461","url":null,"abstract":"<div><p>In 2023, the EBMT Practice harmonization and Guidelines Committee partnered with the EBMT Infection Diseases Working Party (IDWP) to undertake the task of delivering best practice recommendations, aiming to harmonize by expert consensus, the already existing definitions and future epidemiological and clinical studies among centers of the EBMT network. To attain this objective, a group of experts in the field was convened. The workgroup identified and discussed some critical aspects in definitions of community-acquired respiratory viruses (CARV) and adenovirus (ADV) infections in recipient of hematopoietic cell transplant (HCT). The methodology involved literature review and expert consensus. For CARV, expert consensus focused on defining infection severity, infection duration, and establishing criteria for lower respiratory tract disease (LRTD). For ADV, the expert consensus focused on surveillance methods and the definitions of ADV infection, certainty levels of disease, response to treatment, and attributable mortality. This consensus workshop provided indications to EBMT community aimed at facilitating data collection and consistency in the EBMT registry for respiratory viral infectious complications.</p></div>","PeriodicalId":54260,"journal":{"name":"Current Research in Translational Medicine","volume":"72 3","pages":"Article 103461"},"PeriodicalIF":3.2,"publicationDate":"2024-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2452318624000242/pdfft?md5=796a9d025f81cfdf1a3fc56236e60f79&pid=1-s2.0-S2452318624000242-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141713168","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Genetic variants of dectin-1 and their antifungal immunity impact in hematologic malignancies: A comprehensive systematic review","authors":"Mojtaba Aghaei ,&nbsp;Reyhane Khademi ,&nbsp;Mohammad Ali Jalali Far ,&nbsp;Seyed Sobhan Bahreiny ,&nbsp;Amir Hossein Mahdizade ,&nbsp;Nasrin Amirrajab","doi":"10.1016/j.retram.2024.103460","DOIUrl":"10.1016/j.retram.2024.103460","url":null,"abstract":"<div><h3>Background</h3><p>Fungal infections pose a significant threat to individuals with hematologic malignancies due to compromised immune systems. Dectin-1, a pivotal pattern recognition receptor, plays a central role in antifungal immune responses. Understanding its genetic variants' impact is crucial for advancing personalized therapeutic approaches.</p></div><div><h3>Methods</h3><p>Employing systematic review methods, studies were meticulously selected and assessed for relevance. Data extraction encompassed Dectin-1 genetic variants, antifungal immune responses, and disease outcomes.</p></div><div><h3>Results</h3><p>Findings unveiled a complex relationship between Dectin-1 genetic variants and antifungal immunity in hematologic malignancies. Variable associations emerged, influencing susceptibility to fungal infections and disease prognosis. Moreover, implications for treatment outcomes were explored, suggesting potential avenues for tailored interventions.</p></div><div><h3>Conclusions</h3><p>This systematic review underscores the need for further investigation into the precise influence of Dectin-1 genetic variants on antifungal immunity and disease progression in hematologic malignancies. Insights gained could pave the way for personalized therapeutic strategies, optimizing infection prevention and malignancy management. By delving into the intricate connections between genetic nuances, immune responses, and clinical trajectories, this review contributes to the ongoing discourse surrounding hematologic malignancies, fungal infections, and their multifaceted interplay.</p></div>","PeriodicalId":54260,"journal":{"name":"Current Research in Translational Medicine","volume":"72 4","pages":"Article 103460"},"PeriodicalIF":3.2,"publicationDate":"2024-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141710167","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":"Reactive hemophagocytic lymphohistiocytosis: Epidemiological, clinico-biological and etiological profile","authors":"Fatma Abida ,&nbsp;Raida Ben Salah ,&nbsp;Mourad Chaari ,&nbsp;Makram Koubaa ,&nbsp;Mounir Ben Jemaa ,&nbsp;Henda Elleuch ,&nbsp;Zouheir Bahloul","doi":"10.1016/j.retram.2024.103459","DOIUrl":"10.1016/j.retram.2024.103459","url":null,"abstract":"<div><p>Hemophagocytic lymphohistiocytosis (HLH) is an hyperinflammatory state resulting from increased secretion of proinflammatory cytokines, which are responsible for clinical, biological and cytological manifestations.</p></div><div><h3>Objective</h3><p>The aim of our study is to describe the epidemiological, clinical, biological, etiological and evolutionary profile of HLH in Tunisia.</p></div><div><h3>Methods</h3><p>A retrospective study that involved patients, with images of hemophagocytosis in myelograms analyzed at the laboratory of biological hematology of the University Hospital \"Hédi-Chaker\" of Sfax-Tunisia, followed at these departments: hematology, internal medicine, department of infectious-diseases and department of gastroenterology, (June2017- May2021). First, we identified all patients with hemophagocytosis images. Secondly, we selected the patients who fulfilled the diagnostic criteria of the HLH-2004-score.</p></div><div><h3>Results</h3><p>Nineteen patients were included in this study. Nine men and 10 women with a mean age of 37.95 years. Fever was present in all patients. Organomegaly was described in 74% of cases. The most frequent cytopenia was anemia (100%). Hypertriglyceridemia was noted in 79% of cases and hyperferritinemia (&gt; 500 ng/mL) was ubiquitous. In myelogram, 68% of patients had slides showing numerous or very numerous images of hemophagocytosis. The infectious pathology was the most common cause of HLH (42%). No cause was found in 10% of cases. The corticosteroid therapy at a dose of 1 mg/kg/day was prescribed in 89% of our patients. The overall evolution was favorable in 58% of cases. The mortality was not associated with the causal pathology (p=0.218).</p></div><div><h3>Conclusion</h3><p>Secondary HLH is likely to be under-recognized, which contributes to its high morbidity and mortality. Early recognition is crucial for any reasonable attempt at curative therapy.</p></div>","PeriodicalId":54260,"journal":{"name":"Current Research in Translational Medicine","volume":"72 4","pages":"Article 103459"},"PeriodicalIF":3.2,"publicationDate":"2024-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141604504","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}