Mediterranean Journal of Hematology and Infectious Diseases最新文献

{"title":"Association of Rheumatoid Arthritis with Glucose-6-Phosphate Dehydrogenase Deficiency: Results from a Case-Control Study.","authors":"Maria Pina Dore, Giovanni Mario Pes, Sandro Mereu, Jessica Piroddu, Lorenzo Cavagna, Gian Luca Erre","doi":"10.4084/MJHID.2024.056","DOIUrl":"10.4084/MJHID.2024.056","url":null,"abstract":"","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11232680/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141565564","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":"Hodgkin Lymphoma in Children: A 16-year Experience at the Children's Welfare Teaching Hospital of Baghdad, Iraq.","authors":"Anna Maria Testi, Mazin Faisal Al-Jadiry, Maria Luisa Moleti, Stefania Uccini, Amir Fadhil Al-Darraij, Raghad Majid Al-Saeed, Hasanein Habeeb Ghali, Ahmed Hatem Sabhan, Samaher Abdulrazzaq Fadhil, Safaa Abdulelah Al-Badri, Adil Rabeea Alsaadawi, Ameer Dh Hameedi, Manhal Hashim Shanshal, Yasir Saadoon Al-Agele, Fatimah Abdul Ridha Al-Saffar, Nihal Khalid Yaseen, Alfonso Piciocchi, Giovanni Marsili, Salma Abbas Al-Hadad","doi":"10.4084/MJHID.2024.053","DOIUrl":"10.4084/MJHID.2024.053","url":null,"abstract":"<p><strong>Background: </strong>Childhood Hodgkin lymphoma (HL) is an eminently curable disease. Good outcomes can be achieved even in resource-limited settings, and the focus is increasingly on limiting long-term toxicity. Contemporary treatment incorporates a risk-stratified, response-adapted approach using multiagent chemotherapy with/without low-dose radiotherapy. Many developing countries continue to use ABVD-based regimens due to limited acute toxicity, cost, and ease of delivery.</p><p><strong>Objective: </strong>We herein report the outcomes of childhood HL diagnosed and treated in an Iraqi single centre over 16 years.</p><p><strong>Methods: </strong>Children ≤14 years old with biopsy-proven HL were enrolled. Most patients received ABVD chemotherapy or COPP/ABV when Dacarbazine was unavailable. Radiotherapy was not available.</p><p><strong>Results: </strong>Three hundred-three children were consecutively newly diagnosed with HL; 284 were considered eligible for the retrospective analysis (treatment refusals 9; deaths before therapy 5; initially diagnosed of non-Hodgkin lymphoma 5). ABVD scheme was administered to 184 children (65%), COPP/ABV to 83 (29%), and other schemes to the remaining 17 patients. Complete response (CR) was achieved in 277 (98%); 4 (1.4%) showed disease progression, and 1 had stable disease. Four patients in CR abandoned therapy and were in CR at the time of analysis, 2 died from infection. Relapse occurred in 42 patients (15%). The 15-year OS and EFS are 89.7% and 70.3%, respectively.</p><p><strong>Conclusion: </strong>In this single Centre, over 16 years, almost 90% of children suffering from HL survive, despite the numerous limitations in diagnostic procedures, shortage of chemotherapy, no radiotherapy facilities, absence of effective second-line treatments, and finally, therapy abandonment for social and financial reasons.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11232685/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141563745","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":"Expression of Oxidative Stress and Inflammatory Indicators for Coronary Artery Disease in Kawasaki Disease.","authors":"Yang Li, Suwei Lan, Haibo Zhang","doi":"10.4084/MJHID.2024.052","DOIUrl":"10.4084/MJHID.2024.052","url":null,"abstract":"<p><strong>Background: </strong>The paper was to investigate the clinical relevance of oxidative stress (OS) and inflammation-associated targets in coronary artery lesions (CALs) associated with Kawasaki disease (KD).</p><p><strong>Methods: </strong>The clinical data from 455 sufferers diagnosed with KD between February 2021 and June 2023 were gathered and divided into two groups: CAL and NCAL. The regression analysis was conducted to search for independent covariates for CALs related to OS and inflammation. The predictive nomogram was structured according to these risk factors. The properties of the model were estimated using calibration and receiver operating characteristic curves.</p><p><strong>Results: </strong>The levels of CRP, IL-6, PLT count, ESR, ox-HDL, MDA, and PLR were more elevated in CAL patients with KD; interestingly, HDL and superoxide dismutase (SOD) were low in the CAL group. Ascension of CRP, IL-6, ESR, ox-HDL, MDA, and PLR, and diminution of HDL and SOD were considered independent risk factors. The nomogram constructed using these factors demonstrated a satisfactory calibration degree and discriminatory power, with an area under the curve of 0.812. In the verification set, the area under the curve was found to be 0.799.</p><p><strong>Conclusion: </strong>The model was established according to 8 OS and inflammation-associated risk factors bound up with CALs in KD sufferers. It may be a usable approach for early diagnosis of CALs in KD.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11232689/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141563817","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":"Atypical Differentiation Syndrome with Sudden Vision Loss in Acute Promyelocytic Leukemia Treated with ATRA plus ATO: a Clinical Case.","authors":"Eleonora Boscaro, Marco Cerrano, Tommaso Tibaldi, Carlotta Zavatto, Michele Reibaldi, Roberto Freilone, Irene Urbino, Ernesta Audisio, Ilaria Cattani","doi":"10.4084/MJHID.2024.063","DOIUrl":"10.4084/MJHID.2024.063","url":null,"abstract":"","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11232687/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141565565","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":"CAR-T Cells in Chronic Lymphocytic Leukemia.","authors":"Ugo Testa, Elvira Pelosi, Germana Castelli, Alberto Fresa, Luca Laurenti","doi":"10.4084/MJHID.2024.045","DOIUrl":"10.4084/MJHID.2024.045","url":null,"abstract":"<p><p>The treatment outcomes of patients with chronic lymphocytic leukemia (CLL) have considerably improved with the introduction of targeted therapies based on Bruton kinase inhibitors (BTKIs), venetoclax, and anti-CD20 monoclonal antibodies. However, despite these consistent improvements, patients who become resistant to these agents have poor outcomes and need new and more efficacious therapeutic strategies. Among these new treatments, a potentially curative approach consists of the use of chimeric antigen receptor T (CAR-T) cell therapy, which achieved remarkable success in various B-cell malignancies, including B-cell Non-Hodgkin Lymphomas (NHLs) and B-acute lymphoblastic Leukemia (ALL). However, although CAR-T cells were initially used for the treatment of CLL, their efficacy in CLL patients was lower than in other B-cell malignancies. This review analyses possible mechanisms of these failures, highlighting some recent developments that could offer the perspective of the incorporation of CAR-T cells in treatment protocols for relapsed/refractory CLL patients.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11178044/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141331411","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":"Is It Possible to Predict Tumor Progression Through Genomic Characterization of Monoclonal Gammopathy and Smoldering Multiple Myeloma?","authors":"Ugo Testa, Giuseppe Leone, Elvira Pelosi, Germana Castelli, Valerio De Stefano","doi":"10.4084/MJHID.2024.044","DOIUrl":"10.4084/MJHID.2024.044","url":null,"abstract":"<p><p>The study of monoclonal serum proteins has led to the generation of two major theories: one proposing that individuals who had monoclonal proteins without any symptoms or evidence of end-organ damage have a benign condition, the other one suggesting that some individuals with asymptomatic monoclonal proteins may progress to multiple myeloma and thus are affected by a monoclonal gammopathy of undetermined significance (MGUS). Longitudinal studies of subjects with MGUS have supported the second theory. Subsequent studies have characterized and defined the existence of another precursor of multiple myeloma, smoldering multiple myeloma (SMM), intermediate between MGUS and multiple myeloma. Primary molecular events, chromosome translocations, and chromosome number alterations resulting in hyperploidy, required for multiple myeloma development, are already observed in myeloma precursors. MGUS and SMM are heterogeneous conditions with the presence of tumors with distinct pathogenic phenotypes and clinical outcomes. The identification of MGUS and SMM patients with a molecularly defined high risk of progression to MM offers the unique opportunity of early intervention with a therapeutic approach on a low tumor burden.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11178066/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141331416","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":"Lymphocytes in Patients with Chronic Active Epstein-Barr Virus Disease Exhibited Elevated PD-1/PD-L1 Expression and a Prevailing Th2 Immune Response.","authors":"Kang Sun, Chaofan Wu, Qi Kong, Junxia Hu, Lin Shi, Yubo Pi, Dina Suolitiken, Tingting Cui, Leilei Chen, Xiaodan He, Zhengyang Song, Lin Wu, Jingshi Wang, Zhao Wang","doi":"10.4084/MJHID.2024.037","DOIUrl":"10.4084/MJHID.2024.037","url":null,"abstract":"<p><strong>Background and objectives: </strong>Chronic active Epstein-Barr virus disease (CAEBV) is a proliferative disease of EBV⁺ T or natural killer (NK) cells with an unclear pathogenesis. This study aimed to examine the frequency and exhaustion levels of lymphocyte subsets in patients with CAEBV to further investigate the pathogenesis.</p><p><strong>Methods: </strong>Using flow cytometry, we detected the frequency, expression levels of programmed cell death 1 (PD-1) and programmed death ligand 1 (PD-L1), and EBV infection status of peripheral T subsets and NK cells in patients with CAEBV and healthy individuals.</p><p><strong>Results: </strong>24 patients and 15 healthy individuals were enrolled in this study. Patients showed notably higher expression levels of PD-1 and PD-L1 in peripheral T subsets and NK cells compared to healthy individuals (P < 0.05). EBV⁺ lymphocytes exhibited significantly higher PD-L1 expression levels than EBV^- lymphocytes. Additionally, the frequency of effector memory T (Tem) cells was significantly increased in patients, and the PD-L1 expression level was positively correlated with the EBV load. Besides, helper T cell 2 (Th2) immune bias, also favoring EBV amplification, was found in patients, including increased Th2 cell frequency, enhanced response capacity, and elevated serum levels of associated cytokines. The distribution and PD-1 expression levels of peripheral T subsets returned to normal in patients who responded to PD-1 blockade therapy.</p><p><strong>Conclusions: </strong>The up-regulation of the PD-1/PD-L1 pathway of peripheral T and NK cells and Th2 immune predominance jointly promoted EBV replication and the development of CAEBV. PD-1 blockade therapy reduced the PD-1 expression level of lymphocytes and helped normalize the distribution of the T subsets.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11178049/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141331418","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":"EBV-Related Lymphoproliferative Diseases: A Review in Light of New Classifications.","authors":"Pietro Tralongo, Arianna Bakacs, Luigi Maria Larocca","doi":"10.4084/MJHID.2024.042","DOIUrl":"10.4084/MJHID.2024.042","url":null,"abstract":"<p><p>Epstein-Barr virus (EBV) is a prevalent virus that can be detected in the vast majority of the population. Most people are asymptomatic and remain chronically infected throughout their lifetimes. However, in some populations, EBV has been linked to a variety of B-cell lymphoproliferative disorders (LPDs), such as Burkitt lymphoma, classic Hodgkin lymphoma, and other LPDs. T-cell LPDs have been linked to EBV in part of peripheral T-cell lymphomas, angioimmunoblastic T-cell lymphomas, extranodal nasal natural killer/T-cell lymphomas, and other uncommon histotypes. This article summarizes the current evidence for EBV-associated LPDs in light of the upcoming World Health Organization classification and the 2022 ICC classification.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11178045/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141331414","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":"Clinical Care Pathway and Management of Major Bleeding Associated with Nonvitamin K Antagonist Oral Anticoagulants: A Modified Delphi Consensus from Saudi Arabia and UAE.","authors":"Abdulrahman Al Raizah, Fakhr Alayoubi, Galal Hassan Abdelnaby, Hazzaa Alzahrani, Majid Farraj Bakheet, Mohammed A Alskaini, Rasha Buhumaid, Sameer Al Awadhi, Sara Nooruddin Kazim, Thiagarajan Jaiganesh, Mohamed Hamdy Hussein Naguib, Zohair Al Aseri","doi":"10.4084/MJHID.2024.038","DOIUrl":"10.4084/MJHID.2024.038","url":null,"abstract":"<p><strong>Background: </strong>The nonvitamin K antagonist oral anticoagulants (NOACs) have become the mainstay anticoagulation therapy for patients requiring oral anticoagulants (OACs) in the Gulf Council Cooperation (GCC) countries. The frequency of NOAC-associated major bleeding is expected to increase in the Emergency Department (ED). Nonetheless, we still lack local guidelines and recommendations for bleeding management in the region. The present Delphi-based consensus aims to establish a standardized and evidence-based clinical care pathway for managing NOAC-associated major bleeding in the Kingdom of Saudi Arabia (KSA) and the United Arab Emirates (UAE).</p><p><strong>Methods: </strong>We adopted a three-step modified Delphi method to develop evidence-based recommendations through two voting rounds and an advisory meeting between the two rounds. A panel of 11 experts from the KSA and UAE participated in the consensus development.</p><p><strong>Results: </strong>Twenty-eight statements reached the consensus level. These statements addressed key aspects of managing major bleeding events associated with NOACs, including the increased use of NOAC in clinical practice, clinical care pathways, and treatment options.</p><p><strong>Conclusion: </strong>The present Delphi consensus provides evidence-based recommendations and protocols for the management of NOAC-associated bleeding in the region. Patients with major DOAC-induced bleeding should be referred to a well-equipped ED with standardized management protocols. A multidisciplinary approach is recommended for establishing the association between NOAC use and major bleeding. Treating physicians should have prompt access to specific reversal agents to optimize patient outcomes. Real-world evidence and national guidelines are needed to aid all stakeholders involved in NOAC-induced bleeding management.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11178050/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141331413","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":"Letermovir Primary Cytomegalovirus Prophylaxis in Allogeneic Hematopoietic Cell Transplant Recipients: Real-Life Data from a University Hospital in Argentina.","authors":"Fabián Herrera, Diego Torres, Marcia Querci, Andrés Nicolás Rearte, Elena Temporiti, Leandro Riera, Patricio Duarte, Cristina Videla, Pablo Bonvehí","doi":"10.4084/MJHID.2024.039","DOIUrl":"10.4084/MJHID.2024.039","url":null,"abstract":"<p><strong>Background: </strong>Cytomegalovirus (CMV) infection remains the most common clinically significant infection after allogeneic hematopoietic stem cell transplantation (allo-HCT) and is associated with considerable morbidity and mortality.</p><p><strong>Objectives: </strong>The present study was designed to describe and compare the incidence of untreated CMV reactivation (uCMVr), clinically significant infection (cs-CMVi) and disease (CMVd), as well as CMV-related hospitalization and outcome of allo-HCT patients, either treated with letermovir (LET) primary prophylaxis or managed with preemptive therapy (PET).</p><p><strong>Methods: </strong>This is a prospective observational cohort study of adult CMV seropositive allo-HCT patients who either received primary prophylaxis with LET within the first 100 days after HCT or were managed with PET.</p><p><strong>Results: </strong>The study population comprised 105 patients (28 in the LET group and 77 in the PET group). Compared to the PET group, patients in the LET group received more allo-HCT from alternative donors (54.5% vs. 82.14%, <i>P=0.012</i>). More than half of the patients in both groups were classified as high risk for CMVd. In the LET vs. PET group, cs-CMVi and CMVd developed respectively in 0 vs. 50 (64.94%), <i>P=<0.0001</i>, and 0 vs. 6 (7.79%), <i>P=0.18</i>. In the LET group, uCMVr occurred in 5 (17.8%) and were all considered blips. Hospital admissions related to cs-CMVi or CMVd in the PET group vs. LET group were 47 (61.04%) vs. 0, respectively, <i>P=<0.0001</i>. No differences were observed in 100-day mortality.</p><p><strong>Conclusions: </strong>LET primary prophylaxis proved effective in preventing cs-CMVi and CMVd and reducing hospitalizations in allo-HCT adults. Blips can occur during prophylaxis and do not require LET discontinuation.</p>","PeriodicalId":18498,"journal":{"name":"Mediterranean Journal of Hematology and Infectious Diseases","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11178052/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141331417","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}