Pub Date : 2024-07-01eCollection Date: 2024-01-01DOI: 10.4084/MJHID.2024.061
Michele Bibas, Shayna Sarosiek, Jorge J Castillo
Waldenström macroglobulinemia (WM) is an infrequent variant of lymphoma, classified as a B-cell malignancy identified by the presence of IgM paraprotein, infiltration of clonal, small lymphoplasmacytic B cells in the bone marrow, and the MYD88 L265P mutation, which is observed in over 90% of cases. The direct invasion of the malignant cells into tissues like lymph nodes and spleen, along with the immune response related to IgM, can also lead to various health complications, such as cytopenias, hyperviscosity, peripheral neuropathy, amyloidosis, and Bing-Neel syndrome. Chemoimmunotherapy has historically been considered the preferred treatment for WM, wherein the combination of rituximab and nucleoside analogs, alkylating drugs, or proteasome inhibitors has exhibited notable efficacy in inhibiting tumor growth. Recent studies have provided evidence that Bruton Tyrosine Kinase inhibitors (BTKI), either used independently or in conjunction with other drugs, have been shown to be effective and safe in the treatment of WM. The disease is considered to be non-curable, with a median life expectancy of 10 to 12 years.
{"title":"Waldenström Macroglobulinemia - A State-of-the-Art Review: Part 1: Epidemiology, Pathogenesis, Clinicopathologic Characteristics, Differential Diagnosis, Risk Stratification, and Clinical Problems.","authors":"Michele Bibas, Shayna Sarosiek, Jorge J Castillo","doi":"10.4084/MJHID.2024.061","DOIUrl":"10.4084/MJHID.2024.061","url":null,"abstract":"<p><p>Waldenström macroglobulinemia (WM) is an infrequent variant of lymphoma, classified as a B-cell malignancy identified by the presence of IgM paraprotein, infiltration of clonal, small lymphoplasmacytic B cells in the bone marrow, and the MYD88 L265P mutation, which is observed in over 90% of cases. The direct invasion of the malignant cells into tissues like lymph nodes and spleen, along with the immune response related to IgM, can also lead to various health complications, such as cytopenias, hyperviscosity, peripheral neuropathy, amyloidosis, and Bing-Neel syndrome. Chemoimmunotherapy has historically been considered the preferred treatment for WM, wherein the combination of rituximab and nucleoside analogs, alkylating drugs, or proteasome inhibitors has exhibited notable efficacy in inhibiting tumor growth. Recent studies have provided evidence that Bruton Tyrosine Kinase inhibitors (BTKI), either used independently or in conjunction with other drugs, have been shown to be effective and safe in the treatment of WM. The disease is considered to be non-curable, with a median life expectancy of 10 to 12 years.</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/PMC11232678/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141563751","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}
Pub Date : 2024-07-01eCollection Date: 2024-01-01DOI: 10.4084/MJHID.2024.058
B Altinok Gunes, T Ozkan, A Karadag Gurel, S Dalkilic, N Belder, Z Ozkeserli, H Ozdag, M Beksac, N Sayinalp, A M Yagci, A Sunguroglu
Background: Acute myeloid leukaemia (AML) is a disease of the haematopoietic stem cells(HSCs) that is characterised by the uncontrolled proliferation and impaired differentiation of normal haematopoietic stem/progenitor cells. Several pathways that control the proliferation and differentiation of HSCs are impaired in AML. Activation of the Wnt/beta-catenin signalling pathway has been shown in AML and beta-catenin, which is thought to be the key element of this pathway, has been frequently highlighted. The present study was designed to determine beta-catenin expression levels and beta-catenin-related genes in AML.
Methods: In this study, beta-catenin gene expression levels were determined in 19 AML patients and 3 controls by qRT-PCR. Transcriptome analysis was performed on AML grouped according to beta-catenin expression levels. Differentially expressed genes(DEGs) were investigated in detail using the Database for Annotation Visualisation and Integrated Discovery(DAVID), Gene Ontology(GO), Kyoto Encyclopedia of Genes and Genomes(KEGG), STRING online tools.
Results: The transcriptome profiles of our AML samples showed different molecular signature profiles according to their beta-catenin levels(high-low). A total of 20 genes have been identified as hub genes. Among these, TTK, HJURP, KIF14, BTF3, RPL17 and RSL1D1 were found to be associated with beta-catenin and poor survival in AML. Furthermore, for the first time in our study, the ELOV6 gene, which is the most highly up-regulated gene in human AML samples, was correlated with a poor prognosis via high beta-catenin levels.
Conclusion: It is suggested that the identification of beta-catenin-related gene profiles in AML may help to select new therapeutic targets for the treatment of AML.
背景:急性髓性白血病(AML)是一种造血干细胞疾病,其特征是正常造血干细胞/祖细胞不受控制地增殖和分化受损。在急性髓细胞性白血病中,有几种控制造血干细胞增殖和分化的途径受到损害。在急性髓细胞性白血病中,Wnt/β-catenin信号通路被激活,而β-catenin被认为是这一通路的关键因素,经常被强调。本研究旨在确定 AML 中 beta-catenin 的表达水平和 beta-catenin 相关基因:本研究采用 qRT-PCR 方法测定了 19 例 AML 患者和 3 例对照组的 beta 连环素基因表达水平。根据β-catenin的表达水平对AML分组进行转录组分析。使用注释可视化和综合发现数据库(DAVID)、基因本体(GO)、京都基因和基因组百科全书(KEGG)、STRING在线工具对差异表达基因(DEGs)进行了详细研究:AML样本的转录组图谱根据其β-catenin水平(高-低)显示出不同的分子特征图谱。共有 20 个基因被鉴定为中枢基因。其中,TTK、HJURP、KIF14、BTF3、RPL17和RSL1D1被发现与β-catenin和急性髓细胞性白血病的不良生存率相关。此外,在我们的研究中,ELOV6 基因是人类 AML 样本中最高调的基因,它首次通过高水平的 beta 连环素与不良预后相关:结论:对急性髓细胞性白血病中β-catenin相关基因谱的鉴定有助于选择治疗急性髓细胞性白血病的新靶点。
{"title":"Transcriptome Analysis of Beta-Catenin-Related Genes in CD34+ Haematopoietic Stem and Progenitor Cells from Patients with AML.","authors":"B Altinok Gunes, T Ozkan, A Karadag Gurel, S Dalkilic, N Belder, Z Ozkeserli, H Ozdag, M Beksac, N Sayinalp, A M Yagci, A Sunguroglu","doi":"10.4084/MJHID.2024.058","DOIUrl":"10.4084/MJHID.2024.058","url":null,"abstract":"<p><strong>Background: </strong>Acute myeloid leukaemia (AML) is a disease of the haematopoietic stem cells(HSCs) that is characterised by the uncontrolled proliferation and impaired differentiation of normal haematopoietic stem/progenitor cells. Several pathways that control the proliferation and differentiation of HSCs are impaired in AML. Activation of the Wnt/beta-catenin signalling pathway has been shown in AML and beta-catenin, which is thought to be the key element of this pathway, has been frequently highlighted. The present study was designed to determine beta-catenin expression levels and beta-catenin-related genes in AML.</p><p><strong>Methods: </strong>In this study, beta-catenin gene expression levels were determined in 19 AML patients and 3 controls by qRT-PCR. Transcriptome analysis was performed on AML grouped according to beta-catenin expression levels. Differentially expressed genes(DEGs) were investigated in detail using the Database for Annotation Visualisation and Integrated Discovery(DAVID), Gene Ontology(GO), Kyoto Encyclopedia of Genes and Genomes(KEGG), STRING online tools.</p><p><strong>Results: </strong>The transcriptome profiles of our AML samples showed different molecular signature profiles according to their beta-catenin levels(high-low). A total of 20 genes have been identified as hub genes. Among these, <i>TTK, HJURP, KIF14, BTF3, RPL17</i> and <i>RSL1D1</i> were found to be associated with beta-catenin and poor survival in AML. Furthermore, for the first time in our study, the <i>ELOV6</i> gene, which is the most highly up-regulated gene in human AML samples, was correlated with a poor prognosis via high beta-catenin levels.</p><p><strong>Conclusion: </strong>It is suggested that the identification of beta-catenin-related gene profiles in AML may help to select new therapeutic targets for the treatment of AML.</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/PMC11232677/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141563750","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}
Pub Date : 2024-07-01eCollection Date: 2024-01-01DOI: 10.4084/MJHID.2024.054
Andrea Nunzi, Luigi Della Valle, Elisa Linnea Lindfors Rossi, Giorgia Ranucci, Flavia Mallegni, Federico Moretti, Elisa Meddi, Luca Guarnera, Ilaria Tiravanti, Kristian Taka, Elisa Buzzatti, Fabiana Esposito, Roberto Secchi, Francesca Di Giuliano, Flavia Chirico, Raffaele Palmieri, Luca Maurillo, Francesco Buccisano, Carmelo Gurnari, Giovangiacinto Paterno, Adriano Venditti, Maria Ilaria Del Principe
Background: Identification of latent tuberculosis infection (LTBI) is a critical step of tuberculosis surveillance, especially in low-incidence countries. However, it is limited to situations with a higher probability of developing active disease, e.g., patients with hematological malignancies. According to guidelines, in TB non-endemic countries, no clear screening program is established at diagnosis for patients with acute leukemia (AL). The primary endpoint of this study was to establish the prevalence of LTBI in patients with a diagnosis of AL using QuantiFERON (QFT)-TB. Secondarily, radiological and clinical features driving the increased risk of LTBI were evaluated.
Methods: QFT-TB screening was performed before induction or consolidation in all patients with AL (myeloid and lymphoid) treated at our Institution between October 2019 and August 2023.
Results: We accrued 62 patients, of whom 7 (11,3%) tested positive, without any symptoms or signs of active TB, and 2 (3,2%) resulted as indeterminate. All positive patients started prophylaxis with isoniazid 300 mg daily, while patients whose test was indeterminate did not receive any prophylaxis. Active TB was excluded by imaging, as well as microscopic, cultural, and molecular examination on bronchoalveolar lavage if signs of any infection were detected. During the 46 months of observation, no patients developed TB reactivation.
Conclusions: Despite the low sample size, 1/10 of our patients had prior TB exposure, hinting that LTBI could be more common than expected in Italy. This finding suggests implementing TB screening in the pre-treatment setting, particularly at a time when more active treatments are becoming available also for patients ineligible for intensive chemotherapy.
{"title":"Acute Leukemia and Latent Tuberculosis Infection in Italy: Quantiferon-Tb Test Screening in a Low Tuberculosis Incidence Country.","authors":"Andrea Nunzi, Luigi Della Valle, Elisa Linnea Lindfors Rossi, Giorgia Ranucci, Flavia Mallegni, Federico Moretti, Elisa Meddi, Luca Guarnera, Ilaria Tiravanti, Kristian Taka, Elisa Buzzatti, Fabiana Esposito, Roberto Secchi, Francesca Di Giuliano, Flavia Chirico, Raffaele Palmieri, Luca Maurillo, Francesco Buccisano, Carmelo Gurnari, Giovangiacinto Paterno, Adriano Venditti, Maria Ilaria Del Principe","doi":"10.4084/MJHID.2024.054","DOIUrl":"10.4084/MJHID.2024.054","url":null,"abstract":"<p><strong>Background: </strong>Identification of latent tuberculosis infection (LTBI) is a critical step of tuberculosis surveillance, especially in low-incidence countries. However, it is limited to situations with a higher probability of developing active disease, e.g., patients with hematological malignancies. According to guidelines, in TB non-endemic countries, no clear screening program is established at diagnosis for patients with acute leukemia (AL). The primary endpoint of this study was to establish the prevalence of LTBI in patients with a diagnosis of AL using QuantiFERON (QFT)-TB. Secondarily, radiological and clinical features driving the increased risk of LTBI were evaluated.</p><p><strong>Methods: </strong>QFT-TB screening was performed before induction or consolidation in all patients with AL (myeloid and lymphoid) treated at our Institution between October 2019 and August 2023.</p><p><strong>Results: </strong>We accrued 62 patients, of whom 7 (11,3%) tested positive, without any symptoms or signs of active TB, and 2 (3,2%) resulted as indeterminate. All positive patients started prophylaxis with isoniazid 300 mg daily, while patients whose test was indeterminate did not receive any prophylaxis. Active TB was excluded by imaging, as well as microscopic, cultural, and molecular examination on bronchoalveolar lavage if signs of any infection were detected. During the 46 months of observation, no patients developed TB reactivation.</p><p><strong>Conclusions: </strong>Despite the low sample size, 1/10 of our patients had prior TB exposure, hinting that LTBI could be more common than expected in Italy. This finding suggests implementing TB screening in the pre-treatment setting, particularly at a time when more active treatments are becoming available also for patients ineligible for intensive chemotherapy.</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/PMC11232683/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141563816","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}
Pub Date : 2024-07-01eCollection Date: 2024-01-01DOI: 10.4084/MJHID.2024.060
Silvia Maria Trisolini, Alessandro Laganà, Saveria Capria
Immune thrombotic thrombocytopenic purpura (iTTP) is a life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, thrombocytopenia, and ischemic end-organ injury due to microvascular platelet-rich thrombi. iTTP pathophysiology is based on a severe ADAMTS13 deficiency, the specific von Willebrand factor (vWF)-cleaving protease, due to anti-ADAMTS13 autoantibodies. Early diagnosis and treatment reduce the mortality. Frontline therapy includes daily plasma exchange (PEX) with fresh frozen plasma replacement and immunosuppression with corticosteroids. Caplacizumab has recently been added to frontline therapy. Caplacizumab is a nanobody that binds to the A1 domain of vWF, blocking the interaction of ultra-large vWF multimers with the platelet and thereby preventing the formation of platelet-rich thrombi. Caplacizumab reduces mortality due to ischemic events, refractoriness, and exacerbations after PEX discontinuation. Until now, the criteria for response to treatment mainly took into account the normalization of platelet count and discontinuation of PEX; with the use of caplacizumab leading to rapid normalization of platelet count, it has been necessary to redefine the response criteria, taking into account also the underlying autoimmune disease. Monitoring of ADAMTS13 activity is important to identify cases with a low value of activity (<10IU/L), requiring the optimization of immunosuppressive therapy with the addition of Rituximab. Rituximab is effective in patients with refractory disease or relapsing disease. Currently, the use of Rituximab has expanded, both in frontline treatment and during follow-up, as a pre-emptive approach. Some patients do not achieve ADAMTS13 remission following the acute phase despite steroids and rituximab treatment, requiring an individualized immunosuppressive approach to prevent clinical relapse. In iTTP, there is an increased risk of venous thrombotic events (VTEs) as well as arterial thrombotic events, and most occur after platelet normalization. Until now, there has been no consensus on the use of pharmacological thromboprophylaxis in patients on caplacizumab because the drug is known to increase bleeding risk.
{"title":"Immune Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis, Therapy and Open Issues.","authors":"Silvia Maria Trisolini, Alessandro Laganà, Saveria Capria","doi":"10.4084/MJHID.2024.060","DOIUrl":"10.4084/MJHID.2024.060","url":null,"abstract":"<p><p>Immune thrombotic thrombocytopenic purpura (iTTP) is a life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, thrombocytopenia, and ischemic end-organ injury due to microvascular platelet-rich thrombi. iTTP pathophysiology is based on a severe ADAMTS13 deficiency, the specific von Willebrand factor (vWF)-cleaving protease, due to anti-ADAMTS13 autoantibodies. Early diagnosis and treatment reduce the mortality. Frontline therapy includes daily plasma exchange (PEX) with fresh frozen plasma replacement and immunosuppression with corticosteroids. Caplacizumab has recently been added to frontline therapy. Caplacizumab is a nanobody that binds to the A1 domain of vWF, blocking the interaction of ultra-large vWF multimers with the platelet and thereby preventing the formation of platelet-rich thrombi. Caplacizumab reduces mortality due to ischemic events, refractoriness, and exacerbations after PEX discontinuation. Until now, the criteria for response to treatment mainly took into account the normalization of platelet count and discontinuation of PEX; with the use of caplacizumab leading to rapid normalization of platelet count, it has been necessary to redefine the response criteria, taking into account also the underlying autoimmune disease. Monitoring of ADAMTS13 activity is important to identify cases with a low value of activity (<10IU/L), requiring the optimization of immunosuppressive therapy with the addition of Rituximab. Rituximab is effective in patients with refractory disease or relapsing disease. Currently, the use of Rituximab has expanded, both in frontline treatment and during follow-up, as a pre-emptive approach. Some patients do not achieve ADAMTS13 remission following the acute phase despite steroids and rituximab treatment, requiring an individualized immunosuppressive approach to prevent clinical relapse. In iTTP, there is an increased risk of venous thrombotic events (VTEs) as well as arterial thrombotic events, and most occur after platelet normalization. Until now, there has been no consensus on the use of pharmacological thromboprophylaxis in patients on caplacizumab because the drug is known to increase bleeding risk.</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/PMC11232686/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141563747","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}
Pub Date : 2024-07-01eCollection Date: 2024-01-01DOI: 10.4084/MJHID.2024.062
Ugo Testa, Elvira Pelosi, Germana Castelli, Giuseppe Leone
Multiple myeloma (MM) is a disorder of the monoclonal plasma cells and is the second most common hematologic malignancy. MM initiation and progression are dependent upon complex genomic abnormalities. The current pathogenic model of MM includes two types of primary events, represented by chromosome translocations or chromosome number alterations resulting in hyperdiploidy. These primary molecular events are observed both in MM and in monoclonal gammopathy, its premalignant precursor. Subsequent genetic events allow the progression of monoclonal gammopathy to MM and, together with primary events, contribute to the genetic complexity and heterogeneity of MM. Newer therapies have considerably improved patient outcomes; however, MM remains an incurable disease and most patients experience multiple relapses. The dramatic progresses achieved in the analysis of the heterogeneous molecular features of different MM patients allowed a comprehensive molecular classification of MM and the definition of an individualized prognostic model to predict an individual MM patient's response to different therapeutic options. Despite these progresses, prognostic models fail to identify a significant proportion of patients destined to early relapse. Treatment strategies are increasingly. Based on disease biology, trials are enriched for high-risk MMs, whose careful definition and categorization requires DNA sequencing studies.
多发性骨髓瘤(MM)是一种单克隆浆细胞疾病,是第二大最常见的血液系统恶性肿瘤。多发性骨髓瘤的发生和发展取决于复杂的基因组异常。目前 MM 的致病模式包括两类原发性事件,即染色体易位或染色体数目改变导致的超二倍体。这些原发性分子事件在 MM 及其恶性前体单克隆性腺病中都可观察到。随后发生的遗传事件使单克隆丙种球蛋白病发展为 MM,并与原发事件一起导致 MM 遗传的复杂性和异质性。新疗法大大改善了患者的预后,但 MM 仍是一种无法治愈的疾病,大多数患者会经历多次复发。在分析不同 MM 患者的异质性分子特征方面取得的巨大进步,使得 MM 的分子分类得以全面展开,并定义了个体化预后模型,以预测 MM 患者对不同治疗方案的反应。尽管取得了这些进展,但预后模型仍无法识别相当一部分注定会早期复发的患者。治疗策略越来越多。根据疾病生物学原理,对高危 MM 进行了大量试验,而对高危 MM 的仔细定义和分类需要进行 DNA 测序研究。
{"title":"Recent Advances in The Definition of the Molecular Alterations Occurring in Multiple Myeloma.","authors":"Ugo Testa, Elvira Pelosi, Germana Castelli, Giuseppe Leone","doi":"10.4084/MJHID.2024.062","DOIUrl":"10.4084/MJHID.2024.062","url":null,"abstract":"<p><p>Multiple myeloma (MM) is a disorder of the monoclonal plasma cells and is the second most common hematologic malignancy. MM initiation and progression are dependent upon complex genomic abnormalities. The current pathogenic model of MM includes two types of primary events, represented by chromosome translocations or chromosome number alterations resulting in hyperdiploidy. These primary molecular events are observed both in MM and in monoclonal gammopathy, its premalignant precursor. Subsequent genetic events allow the progression of monoclonal gammopathy to MM and, together with primary events, contribute to the genetic complexity and heterogeneity of MM. Newer therapies have considerably improved patient outcomes; however, MM remains an incurable disease and most patients experience multiple relapses. The dramatic progresses achieved in the analysis of the heterogeneous molecular features of different MM patients allowed a comprehensive molecular classification of MM and the definition of an individualized prognostic model to predict an individual MM patient's response to different therapeutic options. Despite these progresses, prognostic models fail to identify a significant proportion of patients destined to early relapse. Treatment strategies are increasingly. Based on disease biology, trials are enriched for high-risk MMs, whose careful definition and categorization requires DNA sequencing studies.</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/PMC11232684/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141563749","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}
Pub Date : 2024-07-01eCollection Date: 2024-01-01DOI: 10.4084/MJHID.2024.059
Guiping Liao, Ting Ting Lu, Changqing Wei, Bei Bei Yang, Manlv Wei, Qiuying Huang, Wuxia Qian, Xiaolin Yin
{"title":"A Case of Congenital Dyserythropoietic Anemia Masked by Hemoglobin H Disease.","authors":"Guiping Liao, Ting Ting Lu, Changqing Wei, Bei Bei Yang, Manlv Wei, Qiuying Huang, Wuxia Qian, Xiaolin Yin","doi":"10.4084/MJHID.2024.059","DOIUrl":"10.4084/MJHID.2024.059","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/PMC11232676/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141563815","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}
Pub Date : 2024-07-01eCollection Date: 2024-01-01DOI: 10.4084/MJHID.2024.055
Matteo Molica, Luca Maurillo, Marco Rossi, Massimo Breccia, Carla Mazzone, Paolo de Fabritiis, Salvatore Perrone
{"title":"Is There a Better Therapeutic Time Window from Diagnosis to Treatment for Elderly Acute Myeloid Leukemia Patients Receiving Hypomethylating Agents?","authors":"Matteo Molica, Luca Maurillo, Marco Rossi, Massimo Breccia, Carla Mazzone, Paolo de Fabritiis, Salvatore Perrone","doi":"10.4084/MJHID.2024.055","DOIUrl":"10.4084/MJHID.2024.055","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/PMC11232675/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141565566","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}
Pub Date : 2024-07-01eCollection Date: 2024-01-01DOI: 10.4084/MJHID.2024.057
Ingvild Hausberg Sørvoll, Ingvild Jenssen Lægreid, Tom Sollid, Maria Therese Ahlen, Silje Johansen, Håkon Reikvam
{"title":"Low Incidence of Anti-PF4/Heparin Antibodies in Patients with Acute Myelogenous Leukemia.","authors":"Ingvild Hausberg Sørvoll, Ingvild Jenssen Lægreid, Tom Sollid, Maria Therese Ahlen, Silje Johansen, Håkon Reikvam","doi":"10.4084/MJHID.2024.057","DOIUrl":"10.4084/MJHID.2024.057","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/PMC11232681/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141565567","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}
Pub Date : 2024-07-01eCollection Date: 2024-01-01DOI: 10.4084/MJHID.2024.051
Uğur Önal, Deniz Akyol Seyhan, Olcay Buse Ketenoğlu, Merve Mert Vahabi, Dilşah Başkol Elik, Seichan Chousein Memetali, Gamze Şanlıdağ İşbilen, Cansu Bulut Avşar, Arda Kaya, Ayse Uyan-Önal, Nazlıhan Yalçın, Günel Guliyeva, Şükrü Dirik, Oğuzhan Acet, Damla Akdağ, Melike Demir Görür, Osman Bozbıyık, Berk Göktepe, Tufan Gümüş, İlkin Çankayalı, Kubilay Demirağ, Mehmet Uyar, Hilal Sipahi, Huseyin Aytac Erdem, Meltem Işıkgöz Taşbakan, Bilgin Arda, Şöhret Aydemir, Sercan Ulusoy, Oguz Resat Sipahi
Background: This study aimed to evaluate the epidemiology of septic shock (SS) associated with intraabdominal infections (IAI) as well as associated mortality and efficacy of early source control in a tertiary-care educational hospital.
Methods: Patients who had SS with IAI and consulted by Infectious Diseases consultants between December 2013 and October 2022 during night shifts in our centre were analyzed retrospectively.
Results: A total number of 390 patients were included. Overall, 30-day mortality was 42.5% on day 3, while day 14 and 30 mortality rates were 63.3% and 71.3%, respectively. Source control by surgical or percutaneous operation was performed in 123 of 390 cases (31.5%), and the mortality rate was significantly lower in cases that were performed source control at any time during SS (65/123-52.8% vs 213/267-79.8%, p<0.001). In 44 of 123 cases (35.7%), source control was performed during the first 12 hours, and mortality was significantly lower in this group versus others (24/44-54.5% vs 254/346-73.4%, p=0.009). On the other hand, female gender (p<0.001, odds ratio(OR)= 2.943, 95%CI=1.714-5.054), diabetes mellitus (p= 0.014, OR=2.284, 95%CI=1.179-4.424), carbapenem-resistant Gram-negative etiology (p=0.011, OR=4.386, 95%CI=1.398-13.759), SOFA≥10 (p<0.001, OR=3.036, 95%CI=1.802-5.114), lactate >3 mg/dl (p<0.001, OR=2.764, 95%CI=1.562-4.891) and lack of source control (p=0.001, OR=2.796, 95%CI=1.523-5.133) were significantly associated with 30-day mortality in logistic regression analysis.
Conclusion: Source control has a vital importance in terms of mortality rates for IAI-related septic shock patients. Our study underscores the need for additional research, as the present analysis indicates that early source control does not manifest as a protective factor in logistic regression.
{"title":"Importance of Source Control in the Subgroup of Intra-Abdominal Infections for Septic Shock Patients: Analysis of 390 Cases.","authors":"Uğur Önal, Deniz Akyol Seyhan, Olcay Buse Ketenoğlu, Merve Mert Vahabi, Dilşah Başkol Elik, Seichan Chousein Memetali, Gamze Şanlıdağ İşbilen, Cansu Bulut Avşar, Arda Kaya, Ayse Uyan-Önal, Nazlıhan Yalçın, Günel Guliyeva, Şükrü Dirik, Oğuzhan Acet, Damla Akdağ, Melike Demir Görür, Osman Bozbıyık, Berk Göktepe, Tufan Gümüş, İlkin Çankayalı, Kubilay Demirağ, Mehmet Uyar, Hilal Sipahi, Huseyin Aytac Erdem, Meltem Işıkgöz Taşbakan, Bilgin Arda, Şöhret Aydemir, Sercan Ulusoy, Oguz Resat Sipahi","doi":"10.4084/MJHID.2024.051","DOIUrl":"10.4084/MJHID.2024.051","url":null,"abstract":"<p><strong>Background: </strong>This study aimed to evaluate the epidemiology of septic shock (SS) associated with intraabdominal infections (IAI) as well as associated mortality and efficacy of early source control in a tertiary-care educational hospital.</p><p><strong>Methods: </strong>Patients who had SS with IAI and consulted by Infectious Diseases consultants between December 2013 and October 2022 during night shifts in our centre were analyzed retrospectively.</p><p><strong>Results: </strong>A total number of 390 patients were included. Overall, 30-day mortality was 42.5% on day 3, while day 14 and 30 mortality rates were 63.3% and 71.3%, respectively. Source control by surgical or percutaneous operation was performed in 123 of 390 cases (31.5%), and the mortality rate was significantly lower in cases that were performed source control at any time during SS (65/123-52.8% vs 213/267-79.8%, p<0.001). In 44 of 123 cases (35.7%), source control was performed during the first 12 hours, and mortality was significantly lower in this group versus others (24/44-54.5% vs 254/346-73.4%, p=0.009). On the other hand, female gender (p<0.001, odds ratio(OR)= 2.943, 95%CI=1.714-5.054), diabetes mellitus (p= 0.014, OR=2.284, 95%CI=1.179-4.424), carbapenem-resistant Gram-negative etiology (p=0.011, OR=4.386, 95%CI=1.398-13.759), SOFA≥10 (p<0.001, OR=3.036, 95%CI=1.802-5.114), lactate >3 mg/dl (p<0.001, OR=2.764, 95%CI=1.562-4.891) and lack of source control (p=0.001, OR=2.796, 95%CI=1.523-5.133) were significantly associated with 30-day mortality in logistic regression analysis.</p><p><strong>Conclusion: </strong>Source control has a vital importance in terms of mortality rates for IAI-related septic shock patients. Our study underscores the need for additional research, as the present analysis indicates that early source control does not manifest as a protective factor in logistic regression.</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/PMC11232688/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141563748","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}